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.

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

Status

Completed

Phases

Phase 3

Study type

Interventional

Source

ClinicalTrials.gov

Registry ID

NCT00891046

Acronym

β-SPECIFIC 3

Enrollment

270

Registered

2009-04-30

Start date

2009-09-30

Completion date

2014-12-31

Last updated

2019-03-26

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Systemic Juvenile Idiopathic Arthritis

Keywords

Flare, arthritis, IL-1beta antagonist, systemic juvenile idiopathic arthritis, CHAQ, steroids, ACR pediatric response, Systemic juvenile idiopathic arthritis with active flare

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.

Interventions

DRUGCanakinumab

Canakinumab

Study design

Allocation

Intervention model

SINGLE_GROUP

Primary purpose

TREATMENT

Masking

NONE

Eligibility

Sex/Gender

ALL

Age

2 Years to 19 Years

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/

Design outcomes

Primary

Measure	Time frame	Description
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

Measure	Time frame	Description
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.
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%.
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 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.
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.
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.
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.

Countries

Argentina, Austria, Belgium, Brazil, Canada, France, Germany, Greece, Hungary, Israel, Italy, Netherlands, Peru, Poland, Russia, Spain, Sweden, Switzerland, Turkey (Türkiye), United Kingdom, United States

Participant flow

Recruitment details

The study was conducted at 61 centres in 20 countries.

Pre-assignment details

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.

Participants by arm

Arm	Count
ACZ885 Treated: Group 1 (Discontinued From Core Study) 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.	33
ACZ885 Treated: Group 2 (Completed Core Study) 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.	63
ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study) 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.	40
ACZ885 Treated: Group 4 (Other Criteria) 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.	11
ACZ885 Treatment Naive 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.	123
Total	270

Withdrawals & dropouts

Period	Reason	FG000	FG001	FG002	FG003	FG004
Overall Study	Administrative problems	0	0	0	0	1
Overall Study	Adverse Event	4	3	2	0	14
Overall Study	Lost to Follow-up	0	0	0	0	1
Overall Study	Participant no longer required drug	0	2	0	0	1
Overall Study	Protocol Violation	1	1	0	0	1
Overall Study	Unsatisfactory therapeutic effect	5	2	19	2	20
Overall Study	Withdrawal by Subject	2	1	2	1	1

Baseline characteristics

Characteristic	Total	ACZ885 Treatment Naive	ACZ885 Treated: Group 4 (Other Criteria)	ACZ885 Treated: Group 3 (Steroid Taper Failures in Core Study)	ACZ885 Treated: Group 2 (Completed Core Study)	ACZ885 Treated: Group 1 (Discontinued From Core Study)
Age, Customized 12 -- <20 years	80 participants	40 participants	2 participants	10 participants	17 participants	11 participants
Age, Customized ≥ 20 years	2 participants	0 participants	0 participants	0 participants	2 participants	0 participants
Age, Customized 2 -- <4 years	24 participants	18 participants	1 participants	3 participants	2 participants	0 participants
Age, Customized 4 -- <6 years	42 participants	15 participants	5 participants	6 participants	10 participants	6 participants
Age, Customized 6 -- <12 years	122 participants	50 participants	3 participants	21 participants	32 participants	16 participants
Sex: Female, Male Female	156 Participants	75 Participants	5 Participants	23 Participants	34 Participants	19 Participants
Sex: Female, Male Male	114 Participants	48 Participants	6 Participants	17 Participants	29 Participants	14 Participants

Adverse events

Event type	EG000 affected / at risk	EG001 affected / at risk
deaths Total, all-cause mortality	— / —	— / —
other Total, other adverse events	124 / 147	91 / 123
serious Total, serious adverse events	47 / 147	40 / 123

Outcome results

Primary

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

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)

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

Arm	Measure	Group	Value (NUMBER)
ACZ885 Treatment Naive	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	SAEs	40 participants
ACZ885 Treatment Naive	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	Discontinuation due to any SAE	13 participants
ACZ885 Treatment Naive	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	AEs	108 participants
ACZ885 Treatment Naive	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	Treatment Related AEs	44 participants
ACZ885 Treatment Naive	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	Discontinuation due to any AE	14 participants
ACZ885 Treatment Naive	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	Treatment related SAEs	1 participants
ACZ885 Treated	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	Discontinuation due to any AE	18 participants
ACZ885 Treated	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	AEs	137 participants
ACZ885 Treated	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	SAEs	47 participants
ACZ885 Treated	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	Treatment related SAEs	4 participants
ACZ885 Treated	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	Discontinuation due to any SAE	14 participants
ACZ885 Treated	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	Treatment Related AEs	57 participants

Primary

Number of Participants With Anti -ACZ885 Antibodies at Any Visit During the Study

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)

Population: The analysis was performed on the SS population.

Arm	Measure	Value (NUMBER)
ACZ885 Treatment Naive	Number of Participants With Anti -ACZ885 Antibodies at Any Visit During the Study	0 participants
ACZ885 Treated	Number of Participants With Anti -ACZ885 Antibodies at Any Visit During the Study	0 participants

Primary

Number of Participants With Clinically Significant Local Injection Site Reactions During the Study

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)

Population: The analysis was performed in SS population.

Arm	Measure	Group	Value (NUMBER)
ACZ885 Treatment Naive	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	No tolerability reaction	115 participants
ACZ885 Treatment Naive	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	Mild tolerability reaction	6 participants
ACZ885 Treatment Naive	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	Moderate tolerability reaction	2 participants
ACZ885 Treatment Naive	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	Severe tolerability reaction	0 participants
ACZ885 Treated	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	Severe tolerability reaction	0 participants
ACZ885 Treated	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	No tolerability reaction	129 participants
ACZ885 Treated	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	Moderate tolerability reaction	3 participants
ACZ885 Treated	Number of Participants With Clinically Significant Local Injection Site Reactions During the Study	Mild tolerability reaction	15 participants

Primary

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

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%.