β-SPECIFIC 3: An Open-label Extension Study of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis and Active Systemic Manifestations Manifestations and Response Characterization Study in Canakinumab Treatment-naïve Patients With Active SJIA With and Without Fever.

Sponsor

Novartis Pharmaceuticals (Industry)

Overall Status

Completed

CT.gov ID

NCT00891046

Collaborator

Pediatric Rheumatology International Trials Organization (Other)

270

Enrollment

Locations

Arm

Duration (Months)

3.7

Patients Per Site

0.1

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

This open-label extension study will permit patients with Systemic Juvenile Idiopathic Arthritis (SJIA) who previously were responsive to treatment with canakinumab and canakinumab treatment-naïve patients with active SJIA with and without fever to be retreated with 4 mg/kg s.c. every 4 weeks and assessed for continued efficacy and safety until discontinuation or when study CACZ885G2402 is in place at their study center or around March 2013, whichever occurs first. Patients who are steroid-free will be able to taper their canakinumab dose to 2 mg/kg s.c. every 4 weeks.

Condition or Disease	Intervention/Treatment	Phase
Systemic Juvenile Idiopathic Arthritis	Drug: Canakinumab	Phase 3

Study Design

Study Type:

Interventional

Actual Enrollment :

270 participants

Allocation:

N/A

Intervention Model:

Single Group Assignment

Masking:

None (Open Label)

Primary Purpose:

Treatment

Official Title:

An Open-label Extension Study of Canakinumab (ACZ885) in Patients With Systemic Juvenile Idiopathic Arthritis (SJIA) and Active Systemic Manifestations Who Participated in Studies ACZ885G2301 and ACZ885G2305; and Response Characterization Study in Canakinumab Treatment-naïve Patients With Active SJIA With and Without Fever

Study Start Date :

Sep 1, 2009

Actual Primary Completion Date :

Dec 1, 2014

Actual Study Completion Date :

Dec 1, 2014

Arms and Interventions

Arm	Intervention/Treatment
Experimental: Canakinumab Canakinumab	Drug: Canakinumab Canakinumab

Arm

Intervention/Treatment

Experimental: Canakinumab

Canakinumab

Drug: Canakinumab

Canakinumab

Outcome Measures

Primary Outcome Measures

Number of Participants With Adverse Events (AEs), Serious Adverse Events (SAEs), AEs by Severity, AEs Leading to Discontinuation, SAEs Leading to Discontinuation, Treatment Related AEs and SAE [From start of study treatment (Day 1) up to end of follow-up period (Week 271 for ACZ885 treated participants and Week 145 for ACZ885 treatment naive participants)]
An AE was defined as any unfavorable and unintended sign, symptom, or disease temporally associated with the use of study drug, whether or not related to study drug. A SAE was defined as an event which was fatal or life threatening, required or prolonged hospitalization, was significantly or permanently disabling or incapacitating, constituted a congenital anomaly or a birth defect, or encompassed any other clinically significant event that could jeopardize the participants or require medical or surgical intervention to prevent one of the aforementioned outcomes. Treatment related AEs or SAEs were defined as AEs or SAEs that were suspected to be related to study treatment as per investigator.
Number of Participants With Anti -ACZ885 Antibodies at Any Visit During the Study [From start of study treatment (Day 1) up to end of follow-up period (Week 271 for ACZ885 treated participants and Week 145 for ACZ885 treatment naive participants)]
Immunogenicity assessment included determination of anti-canakinumab (ACZ885) antibodies in serum samples using BIAcore system.
Number of Participants With Clinically Significant Local Injection Site Reactions During the Study [From start of study treatment (Day 1) up to end of follow-up period (Week 271 for ACZ885 treated participants and Week 145 for ACZ885 treatment naive participants)]
Local injection site tolerability was assessed on the injection site. Each participant was classified into one of the following four categories: 1. no tolerability reactions at any time during the study, 2. mild reaction observed on at least one occasion but no moderate or severe reactions. 3. moderate reaction observed on at least one occasion but no severe reaction. 4. severe reaction observed on at least one occasion.
Percentage of Participants Previously Treated With Anakinra Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 at Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 1-100 millimeter (mm) visual analog scale (VAS); 2. Participants Global Assessment on a 1-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of C-reactive protein (CRP) and 7. Absence of intermittent fever due to severe juvenile idiopathic arthritis (SJIA) during the preceding week. Response was defined as more than or equal to (≥) 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature less than or equal to (≤) 38 °C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Percentage of Participants Previously Treated With Tocilizumab Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 at Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature ≤ 38 °C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Percentage of Participants Previously Treated With Other Biologics Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 at Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature ≤ 38 °C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.

Secondary Outcome Measures

Percentage of Non--Responders Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of -CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature ≤ 38°C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Percentage of Participants With Minimum Adapted ACR Pediatric ≥ 30 at Baseline Who Achieved Minimum Response of ACR Pediatric 30/50/70/90/100 at Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever in the preceding week (variable 7) and with no more than one variable 1 to 6 worsening by more than 30%. For minimum adapted ACR paediatric scores, the last measurement recorded from the participant's previous study was considered baseline for the current study.
Percentage of Participants Able to Taper Oral Steroid Use or Reached Steroid Free Regimen [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Steroid tapering with oral steroids was allowed if the participant achieved an adapted ACR Paediatric 50 response and had no fever. A participant was considered to have tapered steroids successfully, if the steroid dose was reduced from baseline and the participant did not flare and maintained a minimum adapted ACR Paediatric 30 at the last measurement. A participant was considered to have unsuccessfully tapered steroids if the steroid dose was reduced during the study but dose at last assessment was equal to or greater than dose at baseline or; if steroid dose was reduced but the participant did not maintain a minimum adapted ACR Paediatric 30 at the last measurement.
Number of Participants Who Reduced Their Canakinumab Dose to 2 mg/kg [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
The canakinumab dose could be reduced from 4 mg/kg to 2 mg/kg in participants who were steroid-free, if requested by the treating physician and agreed by the sponsor. For treatment naive participants , dose reduction was allowed after the participant had received 6 months treatment with canakinumab.
Percentage of Participants With Clinical Remission [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Clinical remission was defined as at least 6 months of inactive disease or at least 12 months of inactive disease on medication during the extension period. Participants with inactive disease for at least 6 months, but had loss of inactive disease before 12 months were also determined.
Change From Baseline in Disability, Overall Well-Being and Pain Intensity Scores Based on Child Health Assessment Questionnaire (CHAQ) to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
The CHAQ was used to assess physical ability, overall well- being and pain intensity experienced by participants. The CHAQ (disability and well-being) dimension consisted of 20 multiple choice items concerning difficulty in performing eight common activities of daily living; dressing and grooming, arising, eating, walking, reaching, personal hygiene, gripping and other "activities". Participants were graded for the response in four categories, ranging from 0 (without any difficulty), 1 (with some difficulty), 2 (with much difficulty) and 3 (unable to do). Participant's pain intensity was assessed by parents and adult participants (18-20 years old) on a VAS scale of 0-100 mm (0 mm: no pain to 100: very severe pain). Change from baseline was calculated by using the formula = (post baseline value - baseline value). For both scales, lower scores indicate increased functional ability.
Change From Baseline in Health-Related Quality of Life (HRQoL) Over Time Based on Child Health Questionnaire- Parent Form (CHQ-PF50) to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
The Child Health Questionnaire - Parent Form (CHQ-PF50) instrument was used to measure HRQoL aged 5 to 18 years from a parent's perspective. This 14 concept questionnaire measured physical and psychosocial health of the participants on following points: physical functioning, role/social emotional, role/social behavior, role/social physical, bodily pain, general behavior, mental health, self-esteem, general health perception, change in health, parental impact - emotional, parental impact - time, family activities, and family cohesion. Total score ranged from 1-100. Increase in score represented improvement in overall well being of participants. Change from baseline was calculated by using the formula = (post baseline value - baseline value).
Change From Baseline in EuroQual 5 -Dimension Health Status Questionnaire (EQ-5D) Utility Index and Health State Assessment Scores [EQ Visual Analog Scale (EQ-VAS)] to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
EQ-5D HRQoL tool was used for participants above 12 years and EQ-5D proxy for 8-11 years. EQ-5D index scores range from -0.11 (worst possible health, worse than dead), to 0 (dead) to 1 (perfect health). Utility based EQ-5D questionnaire provides generic measure of health for clinical and economic appraisal based on 2 parts: EQ-5D descriptive system - 5 dimensions each with 3 levels (1:no, 2:moderate, 3:severe problem) on: mobility (1=0, 2=0.069, 3=0.314), self-care (1=0, 2=0.104, 3=0.214), usual activities (1=0, 2=0.036, 3=0.094), pain/discomfort (1=0, 2=0, 3=0.386) and anxiety/depression (1=0, 2=0.071, 3=0.2). EQ-5D Total score= 1-0.081-(score of level 2 in present)-0.269 (if at least one of level 3 presents). EQ-5D total score: 1=high quality of life; -0.59 worst quality of life; and EQ-VAS - record participant's self-rated health on vertical, visual analog scale as '100=Best and 0=Worst imaginable health state'. Positive change from baseline score indicated improved health status.
Change From Baseline in Pediatric Daytime Sleepiness Scale (PDSS) Score to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Sleep patterns in children and adolescents aged between 11 and 15 years were determined using PDSS instrument to evaluate whether canakinumab helps in reducing sleepiness in children with SJIA. Participants were assessed on 8 items of PDSS, on a scale of 0 to 4 (0 - never, 1 - seldom, 2- sometimes, 3 - frequently and 4 - always). The sum of all the items was reported as total score with a range of 0-32. Change from baseline was calculated by using the formula = (post baseline value - baseline value). A positive change from baseline score indicated improvement.
Change From Baseline in Growth Velocity Parameter for Height to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Growth velocity parameter height percentile was determined. Percentile was based on the growth charts smoothed percentile curve released by Centers for Disease control and prevention (CDC) in 2000, by sex and age.
Percentage of Participants With Inactive Disease [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Inactive disease was defined as no joints with active arthritis; no fever (body temperature ≤ 38 degree Celsius); no rheumatoid rash, serositis, splenomegaly, hepatomegaly, or generalized lymphadenopathy attributable to SJIA; normal CRP, and a rating of no disease activity on the Physician's Global Assessment of disease activity (with a best possible score ≤10 mm on the VAS).
Change From Baseline in Growth Velocity Parameters to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Growth velocity parameter weight percentile was determined. Percentile was based on the growth charts smoothed percentile curve released by Centers for Disease control and prevention (CDC) in 2000, by sex and age.
Change From Baseline in Growth Velocity Parameter for BMI to Last Assessment of Study [Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier]
Growth velocity parameter BMI percentile was determined. Percentile was based on the growth charts smoothed percentile curve released by Centers for Disease control and prevention (CDC) in 2000, by sex and age.

Eligibility Criteria

Criteria

Ages Eligible for Study:

2 Years to 19 Years

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

Inclusion criteria:

Patients from study CACZ885G2305 or CACZ885G2301 who achieved an adapted ACR pediatric 30 response 15 days after their initial dose of canakinumab but clinically deteriorated afterwards or a minimum ACR Pediatric 30 response was not maintained after Day 15 and intervention is deemed necessary by the investigator, or Patients in study CACZ885G2301 who are not eligible to enter Part II (withdrawal part) because they were not able to meet the corticosteroid entry criteria , or Responder patients in Part I or Part II who had not flared when CACZ885G2301 was stopped, or CACZ885G2301 patients who were responders in Part I but experienced a flare in Part II.
Treatment-naïve patients need to meet the following criteria:
Confirmed diagnosis of systemic juvenile idiopathic arthritis as per ILAR definition that must have occurred at least 2 months prior to enrollment with onset of disease < 16 years of age
Male and female patients aged ≥ 2 to < 20 years of age
Active disease at the time of enrollment defined as having 2 or more of the following:
Documented spiking, intermittent fever (body temperature > 38°C) for at least 1 day during the screening period and within 1 week before first canakinumab dose
At least 2 joints with active arthritis
AND C-reactive protein (CRP) > 30 mg/L (normal range < 10 mg/L) Rash Serositis Lymphadenopathy Hepatosplenomegaly
Naïve to canakinumab

Other protocol-defined inclusion criteria may apply

Exclusion criteria:

History of allergy or hypersensitivity to study drug
With active or recurrent bacterial, fungal or viral infections at time of enrollment

Other protocol inclusion/exclusion criteria may apply

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
1	Novartis Investigative Site	Los Angeles	California	United States	90027
2	Novartis Investigative Site	Louisville	Kentucky	United States	40202
3	Novartis Investigative Site	Boston	Massachusetts	United States	02111
4	Novartis Investigative Site	Cincinnati	Ohio	United States	45229
5	Novartis Investigative Site	Columbus	Ohio	United States	43205
6	Novartis Investigative Site	Portland	Oregon	United States	97232
7	Novartis Investigative Site	Caba	Buenos Aires	Argentina	C1270AAN
8	Novartis Investigative Site	Vienna		Austria	A-1090
9	Novartis Investigative Site	Bruxelles		Belgium	1200
10	Novartis Investigative Site	Gent		Belgium	9000
11	Novartis Investigative Site	Laeken		Belgium	1020
12	Novartis Investigative Site	Leuven		Belgium	3000
13	Novartis Investigative Site	Curitiba	PR	Brazil	80060-900
14	Novartis Investigative Site	Rio de Janeiro	RJ	Brazil	20551-030
15	Novartis Investigative Site	Rio de Janeiro	RJ	Brazil	21941-912
16	Novartis Investigative Site	São Paulo	SP	Brazil	04023-900
17	Novartis Investigative Site	Vancouver	British Columbia	Canada
18	Novartis Investigative Site	Toronto	Ontario	Canada	M5G 1X8
19	Novartis Investigative Site	Montreal	Quebec	Canada	H3T 1C5
20	Novartis Investigative Site	Le Kremlin Bicetre		France	94275
21	Novartis Investigative Site	Lyon		France	69677
22	Novartis Investigative Site	Paris cedex 15		France	75015
23	Novartis Investigative Site	Strasbourg		France	67098
24	Novartis Investigative Site	Bad Bramstedt		Germany	24576
25	Novartis Investigative Site	Berlin		Germany	13125
26	Novartis Investigative Site	Berlin		Germany	13353
27	Novartis Investigative Site	Freiburg		Germany	79106
28	Novartis Investigative Site	Garmisch-Partenkirchen		Germany	82467
29	Novartis Investigative Site	Gießen		Germany	35392
30	Novartis Investigative Site	Hamburg		Germany	20246
31	Novartis Investigative Site	Hamburg		Germany	22081
32	Novartis Investigative Site	Hannover		Germany	30625
33	Novartis Investigative Site	Heidelberg		Germany	69120
34	Novartis Investigative Site	Muenster		Germany	48149
35	Novartis Investigative Site	Saint Augustin		Germany	53757
36	Novartis Investigative Site	Tübingen		Germany	72076
37	Novartis Investigative Site	Ampelokipi	GR	Greece	115 27
38	Novartis Investigative Site	Thessaloniki	GR	Greece	546 39
39	Novartis Investigative Site	Goudi- Athens		Greece	115 27
40	Novartis Investigative Site	Budapest		Hungary	1094
41	Novartis Investigative Site	Haifa		Israel	31096
42	Novartis Investigative Site	Kfar-Sava		Israel	4428164
43	Novartis Investigative Site	Petach-Tikva		Israel	49202
44	Novartis Investigative Site	Ramat Gan		Israel	5266202
45	Novartis Investigative Site	Rehovot		Israel	76100
46	Novartis Investigative Site	Firenze	FI	Italy	50132
47	Novartis Investigative Site	Genova	GE	Italy	16147
48	Novartis Investigative Site	Milano	MI	Italy	20100
49	Novartis Investigative Site	Milano	MI	Italy	20122
50	Novartis Investigative Site	Napoli		Italy	80131
51	Novartis Investigative Site	Utrecht		Netherlands	3584 EA
52	Novartis Investigative Site	Breña	Lima	Peru	05
53	Novartis Investigative Site	Warszawa		Poland	02-637
54	Novartis Investigative Site	Moscow		Russian Federation	115522
55	Novartis Investigative Site	Moscow		Russian Federation	119991
56	Novartis Investigative Site	Saint-Petersburg		Russian Federation	194100
57	Novartis Investigative Site	Esplugues de Llobregat	Barcelona	Spain	08950
58	Novartis Investigative Site	Valencia	Comunidad Valenciana	Spain	46026
59	Novartis Investigative Site	Madrid		Spain	28009
60	Novartis Investigative Site	Madrid		Spain	28034
61	Novartis Investigative Site	Stockholm		Sweden	171 76
62	Novartis Investigative Site	Lausanne		Switzerland	1011
63	Novartis Investigative Site	Ankara		Turkey	06100
64	Novartis Investigative Site	Balcova / Izmir		Turkey	35340
65	Novartis Investigative Site	Fatih / Istanbul		Turkey	34098
66	Novartis Investigative Site	Istanbul		Turkey	34722
67	Novartis Investigative Site	Bath		United Kingdom	BS2 8BJ
68	Novartis Investigative Site	Birmingham		United Kingdom	B4 6NH
69	Novartis Investigative Site	Liverpool		United Kingdom	L12 2AP
70	Novartis Investigative Site	London		United Kingdom	WC1N 1EH
71	Novartis Investigative Site	Manchester		United Kingdom	M9 2AA
72	Novartis Investigative Site	Newcastle Upon Tyme		United Kingdom	NE4 4LP
73	Novartis Investigative Site	Oxford		United Kingdom	OX3 7LD

Sponsors and Collaborators

Novartis Pharmaceuticals
Pediatric Rheumatology International Trials Organization

Investigators

Study Director: Novartis Pharmaceuticals, Novartis Pharmaceuticals

Study Documents (Full-Text)

None provided.

More Information

Additional Information:

Publications

None provided.

Responsible Party:

Novartis Pharmaceuticals

ClinicalTrials.gov Identifier:

NCT00891046

Other Study ID Numbers:

CACZ885G2301E1
EudraCT: 2008-008008-42

First Posted:

Apr 30, 2009

Last Update Posted:

Mar 26, 2019

Last Verified:

Jul 1, 2018

Keywords provided by Novartis Pharmaceuticals

Flare

arthritis

IL-1beta antagonist

systemic juvenile idiopathic arthritis

CHAQ

steroids

ACR pediatric response

Systemic juvenile idiopathic arthritis with active flare

Additional relevant MeSH terms:

Arthritis

Arthritis, Juvenile

Study Results

Participant Flow

Recruitment Details	The study was conducted at 61 centres in 20 countries.
Pre-assignment Detail	A total of 147 participants from studies CACZ885G2301 (NCT number: NCT00889863) and CACZ885G2305 (NCT number: NCT00886769) and 123 canakinumab treatment- naive participants were enrolled into this extension study.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from Study CACZ885G2301 Part 2 (NCT00889863) due to SJIA flare, received a subcutaneous (s.c.).	Participants who completed Study CACZ885G2301 (NCT00889863), received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in CACZ885G2301 (NCT00889863); received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies CACZ885G2301 (NCT00889863) and CACZ885G2305 (NCT00886769), but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Period Title: Overall Study
STARTED	33	63	40	11	123
COMPLETED	21	54	17	8	84
NOT COMPLETED	12	9	23	3	39

Baseline Characteristics

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive	Total
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment- naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Total of all reporting groups
Overall Participants	33	63	40	11	123	270
Age, Customized (participants) [Number]
2 -- <4 years	0 0%	2 3.2%	3 7.5%	1 9.1%	18 14.6%	24 8.9%
4 -- <6 years	6 18.2%	10 15.9%	6 15%	5 45.5%	15 12.2%	42 15.6%
6 -- <12 years	16 48.5%	32 50.8%	21 52.5%	3 27.3%	50 40.7%	122 45.2%
12 -- <20 years	11 33.3%	17 27%	10 25%	2 18.2%	40 32.5%	80 29.6%
≥ 20 years	0 0%	2 3.2%	0 0%	0 0%	0 0%	2 0.7%
Sex: Female, Male (Count of Participants)
Female	19 57.6%	34 54%	23 57.5%	5 45.5%	75 61%	156 57.8%
Male	14 42.4%	29 46%	17 42.5%	6 54.5%	48 39%	114 42.2%

Outcome Measures

1. Primary Outcome

Title	Number of Participants With Adverse Events (AEs), Serious Adverse Events (SAEs), AEs by Severity, AEs Leading to Discontinuation, SAEs Leading to Discontinuation, Treatment Related AEs and SAE
Description	An AE was defined as any unfavorable and unintended sign, symptom, or disease temporally associated with the use of study drug, whether or not related to study drug. A SAE was defined as an event which was fatal or life threatening, required or prolonged hospitalization, was significantly or permanently disabling or incapacitating, constituted a congenital anomaly or a birth defect, or encompassed any other clinically significant event that could jeopardize the participants or require medical or surgical intervention to prevent one of the aforementioned outcomes. Treatment related AEs or SAEs were defined as AEs or SAEs that were suspected to be related to study treatment as per investigator.
Time Frame	From start of study treatment (Day 1) up to end of follow-up period (Week 271 for ACZ885 treated participants and Week 145 for ACZ885 treatment naive participants)

Outcome Measure Data

Analysis Population Description
The analysis was performed in safety set (SS), defined as all participants who received at least one dose of study drug.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	147
AEs	108 327.3%	137 217.5%
SAEs	40 121.2%	47 74.6%
Discontinuation due to any AE	14 42.4%	18 28.6%
Discontinuation due to any SAE	13 39.4%	14 22.2%
Treatment Related AEs	44 133.3%	57 90.5%
Treatment related SAEs	1 3%	4 6.3%

2. Primary Outcome

Title	Number of Participants With Anti -ACZ885 Antibodies at Any Visit During the Study
Description	Immunogenicity assessment included determination of anti-canakinumab (ACZ885) antibodies in serum samples using BIAcore system.
Time Frame	From start of study treatment (Day 1) up to end of follow-up period (Week 271 for ACZ885 treated participants and Week 145 for ACZ885 treatment naive participants)

Outcome Measure Data

Analysis Population Description
The analysis was performed on the SS population.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	147
Number [participants]	0 0%	0 0%

3. Primary Outcome

Title	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study
Description	Local injection site tolerability was assessed on the injection site. Each participant was classified into one of the following four categories: 1. no tolerability reactions at any time during the study, 2. mild reaction observed on at least one occasion but no moderate or severe reactions. 3. moderate reaction observed on at least one occasion but no severe reaction. 4. severe reaction observed on at least one occasion.
Time Frame	From start of study treatment (Day 1) up to end of follow-up period (Week 271 for ACZ885 treated participants and Week 145 for ACZ885 treatment naive participants)

Outcome Measure Data

Analysis Population Description
The analysis was performed in SS population.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	147
No tolerability reaction	115 348.5%	129 204.8%
Mild tolerability reaction	6 18.2%	15 23.8%
Moderate tolerability reaction	2 6.1%	3 4.8%
Severe tolerability reaction	0 0%	0 0%

4. Primary Outcome

Title	Percentage of Participants Previously Treated With Anakinra Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 at Last Assessment of Study
Description	Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 1-100 millimeter (mm) visual analog scale (VAS); 2. Participants Global Assessment on a 1-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of C-reactive protein (CRP) and 7. Absence of intermittent fever due to severe juvenile idiopathic arthritis (SJIA) during the preceding week. Response was defined as more than or equal to (≥) 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature less than or equal to (≤) 38 °C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analyzed' signifies number of participants with an ACR assessment at the given visit.

Arm/Group Title	ACZ885 Treatment Naive: Group 1	ACZ885 Treatment Naive: Group 2	ACZ885 Treatment Naive: Group 3	ACZ885 Treatment Naive: Group 4
Arm/Group Description	Participants who were canakinumab treatment naive and who discontinued anakinra due to lack of efficacy, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who discontinued anakinra for safety/tolerability reasons, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who discontinued anakinra for safety/tolerability reasons, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who never exposed to anakinra, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	26	12	13	70
ACR ≥30 criteria	80.8 244.8%	91.7 145.6%	100 250%	95.7 870%
ACR ≥ 50 criteria	80.8 244.8%	91.7 145.6%	100 250%	92.9 844.5%
ACR ≥ 70 criteria	76.9 233%	91.7 145.6%	92.3 230.8%	90 818.2%
ACR ≥ 90 criteria	69.2 209.7%	91.7 145.6%	76.9 192.3%	80 727.3%
ACR 100 criteria	57.7 174.8%	91.7 145.6%	69.2 173%	64.3 584.5%

5. Primary Outcome

Title	Percentage of Participants Previously Treated With Tocilizumab Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 at Last Assessment of Study
Description	Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature ≤ 38 °C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analyzed' signifies number of participants with an ACR assessment at the given visit.

Arm/Group Title	ACZ885 Treatment Naive: Group 1	ACZ885 Treatment Naive: Group 2	ACZ885 Treatment Naive: Group 3	ACZ885 Treatment Naive: Group 4
Arm/Group Description	Participants who were canakinumab treatment naive and who discontinued tocilizumab due to lack of efficacy, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who discontinued tocilizumab for safety/tolerability reasons, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who discontinued tocilizumab for safety/tolerability reasons, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who never exposed to tocilizumab, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	26	3	2	90
ACR ≥30 criteria	92.3 279.7%	100 158.7%	100 250%	92.2 838.2%
ACR ≥ 50 criteria	88.5 268.2%	100 158.7%	100 250%	91.1 828.2%
ACR ≥ 70 criteria	88.5 268.2%	100 158.7%	100 250%	86.7 788.2%
ACR ≥ 90 criteria	88.5 268.2%	66.7 105.9%	50 125%	76.7 697.3%
ACR 100 criteria	65.4 198.2%	66.7 105.9%	0 0%	67.8 616.4%

6. Primary Outcome

Title	Percentage of Participants Previously Treated With Other Biologics Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100 at Last Assessment of Study
Description	Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature ≤ 38 °C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analyzed' signifies number of participants with an ACR assessment at the given visit. For arm 'ACZ885 treatment naive: Group 2' there were no participants who had discontinued other biologics due to safety/tolerability issues.

Arm/Group Title	ACZ885 Treatment Naive: Group 1	ACZ885 Treatment Naive: Group 2	ACZ885 Treatment Naive: Group 3	ACZ885 Treatment Naive: Group 4
Arm/Group Description	Participants who were canakinumab treatment- naive and who discontinued other biologics due to lack of efficacy, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who discontinued other biologics for safety/tolerability reasons, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who discontinued other biologics for other reasons, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and who never exposed to other biologics, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	30	0	2	89
ACR ≥30 criteria	96.7 293%	100 158.7%	91.0 227.5%
ACR ≥ 50 criteria	93.3 282.7%	50 79.4%	91.0 227.5%
ACR ≥ 70 criteria	86.7 262.7%	50 79.4%	88.8 222%
ACR ≥ 90 criteria	83.3 252.4%	50 79.4%	77.5 193.8%
ACR 100 criteria	60 181.8%	50 79.4%	68.5 171.3%

7. Secondary Outcome

Title	Percentage of Non--Responders Who Achieved Minimum Response of American College of Rheumatology (ACR) Pediatric 30/50/70/90/100
Description	Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of -CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever (i.e. body temperature ≤ 38°C) in the preceding week (variable 7) and with no more than one variable 1 to 6, worsening by more than 30%.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analysed' signifies number of participants with an ACR assessment at the given visit. For arm 'ACZ885 treated: Group 2 (Completed core study)' there were no Non-Responders participants available.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from CACZ885G2301 study Part II (NCT00889863), received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study CACZ885G2301 - Part II (NCT00889863), received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in CACZ885G2301 Study -Part I (NCT00889863); received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies CACZ885G2301 (NCT00889863) and CACZ885G2305 (NCT00886769), but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	17	0	17	6	121
ACR ≥30 criteria	82.4 249.7%	76.5 121.4%	83.3 208.3%	92.6 841.8%
ACR ≥ 50 criteria	82.4 249.7%	70.6 112.1%	83.3 208.3%	90.9 826.4%
ACR ≥ 70 criteria	82.4 249.7%	52.9 84%	83.3 208.3%	87.6 796.4%
ACR ≥ 90 criteria	76.5 231.8%	23.5 37.3%	50 125%	78.5 713.6%
ACR 100 criteria	58.8 178.2%	17.6 27.9%	50 125%	66.1 600.9%

8. Secondary Outcome

Title	Percentage of Participants With Minimum Adapted ACR Pediatric ≥ 30 at Baseline Who Achieved Minimum Response of ACR Pediatric 30/50/70/90/100 at Last Assessment of Study
Description	Adapted ACR Paediatric 30/50/70/90 or 100 was assessed based on following 7 variables: 1.Physician's Global Assessment on a 0-100 mm VAS; 2. Participants Global Assessment on a 0-100 mm VAS; 3. Functional ability; 4. Joints count with active arthritis; 5. Joints count with limitation of motion; 6. Laboratory measure of CRP and 7. Absence of intermittent fever due to SJIA during the preceding week. Response was defined as ≥ 30%/50%/70%/90% or 100% improvement in at least 3 of the response variables 1 to 6, no intermittent fever in the preceding week (variable 7) and with no more than one variable 1 to 6 worsening by more than 30%. For minimum adapted ACR paediatric scores, the last measurement recorded from the participant's previous study was considered baseline for the current study.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analysed' signifies number of participants with an ACR assessment at the given visit.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	16	63	23	5
ACR ≥ 30	100 303%	100 158.7%	91.3 228.3%	100 909.1%
ACR ≥ 50	100 303%	100 158.7%	87 217.5%	100 909.1%
ACR ≥ 70	100 303%	100 158.7%	78.3 195.8%	100 909.1%
ACR ≥ 90	100 303%	100 158.7%	69.6 174%	100 909.1%
ACR ≥ 100	87.5 265.2%	93.7 148.7%	39.1 97.8%	80 727.3%

9. Secondary Outcome

Title	Percentage of Participants Able to Taper Oral Steroid Use or Reached Steroid Free Regimen
Description	Steroid tapering with oral steroids was allowed if the participant achieved an adapted ACR Paediatric 50 response and had no fever. A participant was considered to have tapered steroids successfully, if the steroid dose was reduced from baseline and the participant did not flare and maintained a minimum adapted ACR Paediatric 30 at the last measurement. A participant was considered to have unsuccessfully tapered steroids if the steroid dose was reduced during the study but dose at last assessment was equal to or greater than dose at baseline or; if steroid dose was reduced but the participant did not maintain a minimum adapted ACR Paediatric 30 at the last measurement.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analysed' signifies number of participants who were steroid users at baseline.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	11	9	38	8	71
Steroid free	36.4 110.3%	55.6 88.3%	23.7 59.3%	25 227.3%	33.8 27.5%
Successfully tapered	27.3 82.7%	0 0%	21.1 52.8%	25 227.3%	23.9 19.4%
Unsuccessfully tapered	18.2 55.2%	22.2 35.2%	18.4 46%	25 227.3%	11.3 9.2%
Did not taper	18.2 55.2%	22.2 35.2%	36.8 92%	25 227.3%	31 25.2%

10. Secondary Outcome

Title	Number of Participants Who Reduced Their Canakinumab Dose to 2 mg/kg
Description	The canakinumab dose could be reduced from 4 mg/kg to 2 mg/kg in participants who were steroid-free, if requested by the treating physician and agreed by the sponsor. For treatment naive participants , dose reduction was allowed after the participant had received 6 months treatment with canakinumab.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	33	63	40	11	123
Number [participants]	9 27.3%	29 46%	4 10%	2 18.2%	18 14.6%

11. Secondary Outcome

Title	Percentage of Participants With Clinical Remission
Description	Clinical remission was defined as at least 6 months of inactive disease or at least 12 months of inactive disease on medication during the extension period. Participants with inactive disease for at least 6 months, but had loss of inactive disease before 12 months were also determined.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was done in FAS population. Here 'Number of participants analysed' signifies number of participants with an assessment in the given visit.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	147
At least 6 consecutive months of inactive disease	42.3 128.2%	52.4 83.2%
At least 12 consecutive months of inactive disease	26.8 81.2%	42.9 68.1%
Loss of inactive disease after 6 months	8.9 27%	6.8 10.8%

12. Secondary Outcome

Title	Change From Baseline in Disability, Overall Well-Being and Pain Intensity Scores Based on Child Health Assessment Questionnaire (CHAQ) to Last Assessment of Study
Description	The CHAQ was used to assess physical ability, overall well- being and pain intensity experienced by participants. The CHAQ (disability and well-being) dimension consisted of 20 multiple choice items concerning difficulty in performing eight common activities of daily living; dressing and grooming, arising, eating, walking, reaching, personal hygiene, gripping and other "activities". Participants were graded for the response in four categories, ranging from 0 (without any difficulty), 1 (with some difficulty), 2 (with much difficulty) and 3 (unable to do). Participant's pain intensity was assessed by parents and adult participants (18-20 years old) on a VAS scale of 0-100 mm (0 mm: no pain to 100: very severe pain). Change from baseline was calculated by using the formula = (post baseline value - baseline value). For both scales, lower scores indicate increased functional ability.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	33	63	40	11	121
Disability score	-0.375	0	-0.125	0	-0.7143
Overall well-being score	-18	0	0	-10	-28
Pain Intensity score	-13	0	0	0	-39

13. Secondary Outcome

Title	Change From Baseline in Health-Related Quality of Life (HRQoL) Over Time Based on Child Health Questionnaire- Parent Form (CHQ-PF50) to Last Assessment of Study
Description	The Child Health Questionnaire - Parent Form (CHQ-PF50) instrument was used to measure HRQoL aged 5 to 18 years from a parent's perspective. This 14 concept questionnaire measured physical and psychosocial health of the participants on following points: physical functioning, role/social emotional, role/social behavior, role/social physical, bodily pain, general behavior, mental health, self-esteem, general health perception, change in health, parental impact - emotional, parental impact - time, family activities, and family cohesion. Total score ranged from 1-100. Increase in score represented improvement in overall well being of participants. Change from baseline was calculated by using the formula = (post baseline value - baseline value).
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here 'Number of participants analysed' signifies number of participants with HRQoL assessment in the given visit.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	26	44	29	8	90
CHQ-PF50 physical score	14.0407	0.6959	1.3716	13.9255	18.8758
CHQ-PF50 psychosocial score	3.8815	1.4004	0.7582	11.9798	9.3209

14. Secondary Outcome

Title	Change From Baseline in EuroQual 5 -Dimension Health Status Questionnaire (EQ-5D) Utility Index and Health State Assessment Scores [EQ Visual Analog Scale (EQ-VAS)] to Last Assessment of Study
Description	EQ-5D HRQoL tool was used for participants above 12 years and EQ-5D proxy for 8-11 years. EQ-5D index scores range from -0.11 (worst possible health, worse than dead), to 0 (dead) to 1 (perfect health). Utility based EQ-5D questionnaire provides generic measure of health for clinical and economic appraisal based on 2 parts: EQ-5D descriptive system - 5 dimensions each with 3 levels (1:no, 2:moderate, 3:severe problem) on: mobility (1=0, 2=0.069, 3=0.314), self-care (1=0, 2=0.104, 3=0.214), usual activities (1=0, 2=0.036, 3=0.094), pain/discomfort (1=0, 2=0, 3=0.386) and anxiety/depression (1=0, 2=0.071, 3=0.2). EQ-5D Total score= 1-0.081-(score of level 2 in present)-0.269 (if at least one of level 3 presents). EQ-5D total score: 1=high quality of life; -0.59 worst quality of life; and EQ-VAS - record participant's self-rated health on vertical, visual analog scale as '100=Best and 0=Worst imaginable health state'. Positive change from baseline score indicated improved health status.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here 'Number of participants analyzed' signifies number of participants with EQ-5D assessment in the given visit.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	17	35	22	4	65
EQ- 5D Utility Index	0.204	0	0.069	0.2385	0.228
EQ-VAS	21	0	5	28	30

15. Secondary Outcome

Title	Change From Baseline in Pediatric Daytime Sleepiness Scale (PDSS) Score to Last Assessment of Study
Description	Sleep patterns in children and adolescents aged between 11 and 15 years were determined using PDSS instrument to evaluate whether canakinumab helps in reducing sleepiness in children with SJIA. Participants were assessed on 8 items of PDSS, on a scale of 0 to 4 (0 - never, 1 - seldom, 2- sometimes, 3 - frequently and 4 - always). The sum of all the items was reported as total score with a range of 0-32. Change from baseline was calculated by using the formula = (post baseline value - baseline value). A positive change from baseline score indicated improvement.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here, 'Number of participants analysed' signifies those participants with a value at both baseline and the respective post baseline time point and with an assessment of PDSS score in the given visit..

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)
Arm/Group Description	Participants who discontinued from NCT00889863, received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study NCT00889863, received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in NCT00889863; received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies NCT00889863 and NCT00886769, but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	9	12	5	2
Median (Full Range) [units on a scale]	1	0.5	0	-4.5

16. Secondary Outcome

Title	Change From Baseline in Growth Velocity Parameter for Height to Last Assessment of Study
Description	Growth velocity parameter height percentile was determined. Percentile was based on the growth charts smoothed percentile curve released by Centers for Disease control and prevention (CDC) in 2000, by sex and age.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here, 'Number of participants analyzed' signifies those participants with a value at both baseline and the respective post baseline time point.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	139
Median (Full Range) [Percentile]	-0.01	0

17. Secondary Outcome

Title	Percentage of Participants With Inactive Disease
Description	Inactive disease was defined as no joints with active arthritis; no fever (body temperature ≤ 38 degree Celsius); no rheumatoid rash, serositis, splenomegaly, hepatomegaly, or generalized lymphadenopathy attributable to SJIA; normal CRP, and a rating of no disease activity on the Physician's Global Assessment of disease activity (with a best possible score ≤10 mm on the VAS).
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here 'Number of participants analysed' signifies number of participants with an assessment in the given visit.

Arm/Group Title	ACZ885 Treated: Group 1 (Discontinued From Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treatment Naive
Arm/Group Description	Participants who discontinued from CACZ885G2301 study Part II (NCT00889863), received a subcutaneous (s.c.) injection of canakinumab 4 mg/kg every 4 weeks unless discontinuation occurs. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who completed study CACZ885G2301 - Part II (NCT00889863), received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who failed to taper their steroid dose in CACZ885G2301 Study -Part I (NCT00889863); received an s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who previously received canakinumab treatment in Studies CACZ885G2301 (NCT00889863) and CACZ885G2305 (NCT00886769), but did not fulfill the criteria for Group 1, 2 or 3, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
Measure Participants	33	63	40	11	122
Number [Percentage of participants]	39.4 119.4%	79.4 126%	12.5 31.3%	36.4 330.9%	50.8 41.3%

18. Secondary Outcome

Title	Change From Baseline in Growth Velocity Parameters to Last Assessment of Study
Description	Growth velocity parameter weight percentile was determined. Percentile was based on the growth charts smoothed percentile curve released by Centers for Disease control and prevention (CDC) in 2000, by sex and age.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here, 'Number of participants analyzed' signifies those participants with a value at both baseline and the respective post baseline time point.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	139
Median (Full Range) [Percentile]	0.1	0

19. Secondary Outcome

Title	Change From Baseline in Growth Velocity Parameter for BMI to Last Assessment of Study
Description	Growth velocity parameter BMI percentile was determined. Percentile was based on the growth charts smoothed percentile curve released by Centers for Disease control and prevention (CDC) in 2000, by sex and age.
Time Frame	Baseline up to last assessment (4 years) or date of discontinuation, which ever occurred earlier

Outcome Measure Data

Analysis Population Description
The analysis was performed in FAS population. Here, 'Number of participants analyzed' signifies those participants with a value at both baseline and the respective post baseline time point.

Arm/Group Title	ACZ885 Treatment Naive	ACZ885 Treated
Arm/Group Description	Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.
Measure Participants	123	139
Median (Full Range) [Percentile]	1.08	-0.77

Adverse Events

	ACZ885 Treated		ACZ885 Treatment Naive
Time Frame	From start of study treatment (Day 1) to end of follow-up period (Week 271)
Adverse Event Reporting Description	The analysis was performed in safety set, defined as all subjects who received at least one dose of study drug under this study protocol.

Arm/Group Title	ACZ885 Treated		ACZ885 Treatment Naive
Arm/Group Description	Participants who were responsive to canakinumab in previous studies: NCT00889863 and NCT00886769 and entered into this extension study in Group 1, 2, 3 and 4.		Participants who were canakinumab treatment naive and did not participate in previous canakinumab studies, received a s.c. injection of canakinumab 4 mg/kg every 4 weeks. Canakinumab 2 mg/kg was administered for participants who were able to taper their steroid dose.
All Cause Mortality
	Affected / at Risk (%)	# Events	Affected / at Risk (%)	# Events
Total	/ (NaN)		/ (NaN)
Serious Adverse Events
	ACZ885 Treated		ACZ885 Treatment Naive
	Affected / at Risk (%)	# Events	Affected / at Risk (%)	# Events
Total	47/147 (32%)		40/123 (32.5%)
Blood and lymphatic system disorders
Abdominal lymphadenopathy	0/147 (0%)		1/123 (0.8%)
Anaemia	0/147 (0%)		1/123 (0.8%)
Histiocytosis haematophagic	10/147 (6.8%)		6/123 (4.9%)
Leukopenia	1/147 (0.7%)		2/123 (1.6%)
Lymphadenitis	0/147 (0%)		3/123 (2.4%)
Lymphadenopathy	1/147 (0.7%)		3/123 (2.4%)
Neutropenia	0/147 (0%)		1/123 (0.8%)
Splenomegaly	1/147 (0.7%)		0/123 (0%)
Thrombocytopenia	1/147 (0.7%)		1/123 (0.8%)
Cardiac disorders
Pericardial effusion	0/147 (0%)		1/123 (0.8%)
Pericarditis	1/147 (0.7%)		0/123 (0%)
Gastrointestinal disorders
Abdominal pain	2/147 (1.4%)		1/123 (0.8%)
Abdominal pain upper	0/147 (0%)		1/123 (0.8%)
Colitis	0/147 (0%)		1/123 (0.8%)
Colitis ulcerative	0/147 (0%)		1/123 (0.8%)
Enteritis	0/147 (0%)		1/123 (0.8%)
Gastrointestinal disorder	0/147 (0%)		1/123 (0.8%)
Gastrooesophageal reflux disease	1/147 (0.7%)		0/123 (0%)
Hiatus hernia	1/147 (0.7%)		0/123 (0%)
General disorders
Device dislocation	0/147 (0%)		1/123 (0.8%)
Disease progression	1/147 (0.7%)		0/123 (0%)
Drug ineffective	1/147 (0.7%)		1/123 (0.8%)
Injury associated with device	0/147 (0%)		1/123 (0.8%)
Pyrexia	5/147 (3.4%)		4/123 (3.3%)
Hepatobiliary disorders
Autoimmune hepatitis	1/147 (0.7%)		0/123 (0%)
Hepatitis	2/147 (1.4%)		0/123 (0%)
Hepatitis toxic	0/147 (0%)		1/123 (0.8%)
Hepatocellular injury	0/147 (0%)		1/123 (0.8%)
Hepatomegaly	1/147 (0.7%)		0/123 (0%)
Immune system disorders
Drug hypersensitivity	0/147 (0%)		1/123 (0.8%)
Infections and infestations
Abscess neck	0/147 (0%)		1/123 (0.8%)
Appendicitis	1/147 (0.7%)		0/123 (0%)
Bronchopneumonia	0/147 (0%)		1/123 (0.8%)
Cellulitis	0/147 (0%)		1/123 (0.8%)
Cytomegalovirus hepatitis	1/147 (0.7%)		0/123 (0%)
Cytomegalovirus infection	1/147 (0.7%)		1/123 (0.8%)
Device related sepsis	1/147 (0.7%)		0/123 (0%)
Escherichia urinary tract infection	0/147 (0%)		1/123 (0.8%)
Furuncle	0/147 (0%)		1/123 (0.8%)
Gastroenteritis	4/147 (2.7%)		2/123 (1.6%)
Gastroenteritis salmonella	1/147 (0.7%)		0/123 (0%)
Gastroenteritis yersinia	1/147 (0.7%)		0/123 (0%)
Gastrointestinal viral infection	1/147 (0.7%)		0/123 (0%)
Herpes zoster	1/147 (0.7%)		1/123 (0.8%)
Impetigo	1/147 (0.7%)		0/123 (0%)
Infected bites	0/147 (0%)		1/123 (0.8%)
Influenza	0/147 (0%)		1/123 (0.8%)
Lower respiratory tract infection	1/147 (0.7%)		0/123 (0%)
Lymph node abscess	0/147 (0%)		1/123 (0.8%)
Lymphangitis	0/147 (0%)		1/123 (0.8%)
Meningitis viral	1/147 (0.7%)		0/123 (0%)
Otitis media acute	0/147 (0%)		1/123 (0.8%)
Parvovirus infection	1/147 (0.7%)		0/123 (0%)
Peritonitis	1/147 (0.7%)		0/123 (0%)
Pharyngitis streptococcal	1/147 (0.7%)		0/123 (0%)
Pneumonia	2/147 (1.4%)		3/123 (2.4%)
Pneumonia bacterial	1/147 (0.7%)		0/123 (0%)
Pseudocroup	1/147 (0.7%)		0/123 (0%)
Salmonella sepsis	0/147 (0%)		1/123 (0.8%)
Scarlet fever	1/147 (0.7%)		1/123 (0.8%)
Sepsis	0/147 (0%)		1/123 (0.8%)
Septic shock	1/147 (0.7%)		0/123 (0%)
Sinusitis	1/147 (0.7%)		0/123 (0%)
Staphylococcal sepsis	0/147 (0%)		1/123 (0.8%)
Subcutaneous abscess	2/147 (1.4%)		0/123 (0%)
Tonsillitis streptococcal	2/147 (1.4%)		0/123 (0%)
Toxoplasmosis	1/147 (0.7%)		0/123 (0%)
Varicella	3/147 (2%)		1/123 (0.8%)
Viral upper respiratory tract infection	1/147 (0.7%)		0/123 (0%)
Wound infection	1/147 (0.7%)		0/123 (0%)
Injury, poisoning and procedural complications
Ankle fracture	0/147 (0%)		1/123 (0.8%)
Arteriovenous fistula site complication	0/147 (0%)		1/123 (0.8%)
Fibula fracture	0/147 (0%)		1/123 (0.8%)
Post procedural haemorrhage	0/147 (0%)		1/123 (0.8%)
Road traffic accident	1/147 (0.7%)		0/123 (0%)
Transfusion-related acute lung injury	1/147 (0.7%)		0/123 (0%)
Investigations
Alanine aminotransferase increased	0/147 (0%)		1/123 (0.8%)
Aspartate aminotransferase increased	0/147 (0%)		1/123 (0.8%)
C-reactive protein increased	1/147 (0.7%)		0/123 (0%)
Hepatic enzyme increased	1/147 (0.7%)		1/123 (0.8%)
Liver function test abnormal	1/147 (0.7%)		0/123 (0%)
Serum ferritin increased	1/147 (0.7%)		0/123 (0%)
Transaminases increased	0/147 (0%)		1/123 (0.8%)
Weight decreased	1/147 (0.7%)		0/123 (0%)
Musculoskeletal and connective tissue disorders
Arthralgia	1/147 (0.7%)		1/123 (0.8%)
Intervertebral disc compression	0/147 (0%)		1/123 (0.8%)
Juvenile idiopathic arthritis	14/147 (9.5%)		13/123 (10.6%)
Musculoskeletal chest pain	2/147 (1.4%)		0/123 (0%)
Musculoskeletal disorder	1/147 (0.7%)		0/123 (0%)
Osteoarthritis	1/147 (0.7%)		0/123 (0%)
Osteonecrosis	0/147 (0%)		1/123 (0.8%)
Osteoporosis	1/147 (0.7%)		0/123 (0%)
Polyarthritis	0/147 (0%)		2/123 (1.6%)
Neoplasms benign, malignant and unspecified (incl cysts and polyps)
Anaplastic large cell lymphoma T- and null-cell types	0/147 (0%)		1/123 (0.8%)
Nervous system disorders
Convulsion	0/147 (0%)		1/123 (0.8%)
Migraine	1/147 (0.7%)		0/123 (0%)
Nervous system disorder	0/147 (0%)		1/123 (0.8%)
Paraesthesia	1/147 (0.7%)		0/123 (0%)
Superior sagittal sinus thrombosis	1/147 (0.7%)		0/123 (0%)
Psychiatric disorders
Abnormal behaviour	1/147 (0.7%)		0/123 (0%)
Suicide attempt	1/147 (0.7%)		0/123 (0%)
Renal and urinary disorders
Renal failure acute	1/147 (0.7%)		0/123 (0%)
Reproductive system and breast disorders
Vulvovaginal pain	0/147 (0%)		1/123 (0.8%)
Vulvovaginal pruritus	0/147 (0%)		1/123 (0.8%)
Respiratory, thoracic and mediastinal disorders
Acute interstitial pneumonitis	1/147 (0.7%)		0/123 (0%)
Cough	1/147 (0.7%)		0/123 (0%)
Dyspnoea	1/147 (0.7%)		0/123 (0%)
Skin and subcutaneous tissue disorders
Dermatitis allergic	1/147 (0.7%)		2/123 (1.6%)
Drug reaction with eosinophilia and systemic symptoms	1/147 (0.7%)		0/123 (0%)
Eczema	1/147 (0.7%)		0/123 (0%)
Henoch-Schonlein purpura	0/147 (0%)		1/123 (0.8%)
Ingrowing nail	1/147 (0.7%)		0/123 (0%)
Rash	2/147 (1.4%)		1/123 (0.8%)
Toxic skin eruption	1/147 (0.7%)		0/123 (0%)
Urticaria	0/147 (0%)		1/123 (0.8%)
Other (Not Including Serious) Adverse Events
	ACZ885 Treated		ACZ885 Treatment Naive
	Affected / at Risk (%)	# Events	Affected / at Risk (%)	# Events
Total	124/147 (84.4%)		91/123 (74%)
Ear and labyrinth disorders
Ear pain	10/147 (6.8%)		7/123 (5.7%)
Gastrointestinal disorders
Abdominal pain	21/147 (14.3%)		22/123 (17.9%)
Abdominal pain upper	18/147 (12.2%)		13/123 (10.6%)
Diarrhoea	30/147 (20.4%)		16/123 (13%)
Nausea	17/147 (11.6%)		11/123 (8.9%)
Vomiting	35/147 (23.8%)		18/123 (14.6%)
General disorders
Fatigue	10/147 (6.8%)		3/123 (2.4%)
Pyrexia	38/147 (25.9%)		27/123 (22%)
Infections and infestations
Bronchitis	13/147 (8.8%)		6/123 (4.9%)
Conjunctivitis	8/147 (5.4%)		6/123 (4.9%)
Gastroenteritis	28/147 (19%)		19/123 (15.4%)
Influenza	14/147 (9.5%)		4/123 (3.3%)
Nasopharyngitis	47/147 (32%)		32/123 (26%)
Oral herpes	11/147 (7.5%)		1/123 (0.8%)
Otitis media	11/147 (7.5%)		7/123 (5.7%)
Pharyngitis	15/147 (10.2%)		15/123 (12.2%)
Respiratory tract infection	7/147 (4.8%)		13/123 (10.6%)
Rhinitis	35/147 (23.8%)		26/123 (21.1%)
Tonsillitis	10/147 (6.8%)		7/123 (5.7%)
Upper respiratory tract infection	36/147 (24.5%)		24/123 (19.5%)
Urinary tract infection	8/147 (5.4%)		4/123 (3.3%)
Viral infection	6/147 (4.1%)		11/123 (8.9%)
Viral upper respiratory tract infection	10/147 (6.8%)		4/123 (3.3%)
Injury, poisoning and procedural complications
Arthropod bite	9/147 (6.1%)		12/123 (9.8%)
Contusion	11/147 (7.5%)		0/123 (0%)
Fall	11/147 (7.5%)		7/123 (5.7%)
Ligament sprain	11/147 (7.5%)		5/123 (4.1%)
Investigations
Alanine aminotransferase increased	8/147 (5.4%)		5/123 (4.1%)
Musculoskeletal and connective tissue disorders
Arthralgia	36/147 (24.5%)		25/123 (20.3%)
Arthritis	9/147 (6.1%)		7/123 (5.7%)
Back pain	11/147 (7.5%)		9/123 (7.3%)
Joint swelling	9/147 (6.1%)		5/123 (4.1%)
Juvenile idiopathic arthritis	23/147 (15.6%)		27/123 (22%)
Myalgia	9/147 (6.1%)		6/123 (4.9%)
Neck pain	8/147 (5.4%)		7/123 (5.7%)
Pain in extremity	19/147 (12.9%)		12/123 (9.8%)
Neoplasms benign, malignant and unspecified (incl cysts and polyps)
Skin papilloma	14/147 (9.5%)		4/123 (3.3%)
Nervous system disorders
Headache	30/147 (20.4%)		24/123 (19.5%)
Respiratory, thoracic and mediastinal disorders
Cough	37/147 (25.2%)		28/123 (22.8%)
Epistaxis	10/147 (6.8%)		5/123 (4.1%)
Oropharyngeal pain	36/147 (24.5%)		17/123 (13.8%)
Skin and subcutaneous tissue disorders
Eczema	12/147 (8.2%)		16/123 (13%)
Rash	15/147 (10.2%)		13/123 (10.6%)
Urticaria	7/147 (4.8%)		7/123 (5.7%)

Limitations/Caveats

[Not Specified]

More Information

Certain Agreements

Principal Investigators are NOT employed by the organization sponsoring the study.

The terms and conditions of Novartis' agreements with its investigators may vary. However, Novartis does not prohibit any investigator from publishing. Any publications from a single site are postponed until the publication of the pooled data (i.e, data from all sites) in the clinical trial.

Results Point of Contact

Name/Title	Study Director
Organization	Novartis Pharmaceuticals
Phone	862--778--8300
Email

Responsible Party:

Novartis Pharmaceuticals

ClinicalTrials.gov Identifier:

NCT00891046

Other Study ID Numbers:

CACZ885G2301E1
EudraCT: 2008-008008-42

First Posted:

Apr 30, 2009

Last Update Posted:

Mar 26, 2019

Last Verified:

Jul 1, 2018

β-SPECIFIC 3: An Open-label Extension Study of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis and Active Systemic Manifestations Manifestations and Response Characterization Study in Canakinumab Treatment-naïve Patients With Active SJIA With and Without Fever.

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