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Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis
Mansi Dewoolkar1, Rolando Cimaz2, Pranav Raman Chickermane1
1Department of Pediatrics, Pediatric Rheumatology Clinic, Jaslok Hospital and Research Centre, Mumbai, 400026, India.
Insights
Systemic onset juvenile idiopathic arthritis (s-JIA) patients often face complications, impacting long-term outcomes. Early diagnosis and multidisciplinary care are crucial for managing s-JIA, especially in resource-limited settings.
Area of Science:
- Rheumatology
- Pediatric Immunology
- Clinical Medicine
Background:
- Systemic onset juvenile idiopathic arthritis (s-JIA) is a severe form of childhood arthritis.
- Understanding its disease course, outcomes, and complications is vital for patient management.
- Resource limitations can significantly affect s-JIA patient care and prognosis.
Purpose of the Study:
- To evaluate the disease course, treatment outcomes, and complications in a cohort of s-JIA patients.
- To identify factors influencing functional status and disease remission.
- To assess the impact of complications on s-JIA patient outcomes.
Main Methods:
- An observational study followed 53 s-JIA patients from diagnosis (before Oct 2009) to Sep 2012.
- Clinical examinations, lab tests, and treatment details were recorded every 6-12 weeks.
- Disease course (monocyclic, intermittent, persistent), remission (Wallace criteria), functional status (Steinbrocker), and damage (JADI) were assessed.
Main Results:
- The mean follow-up was 5.5 years; 33 patients experienced disease/drug complications, including infections (30%) and macrophage activation syndrome (9.4%).
- Disease courses were monocyclic (17%), intermittent (58.5%), and persistent (24.5%). Remission rates varied by course (9/9 monocyclic, 17/31 intermittent, 3/13 persistent).
- 96.2% achieved good functional class (I-II), with monocyclic cases faring best. Significant articular damage (JADI-A) was noted in 9 patients.
Conclusions:
- The outcome for s-JIA patients is influenced by complications from chronic disease and steroid overuse.
- Resource limitations, including access to early diagnosis, multidisciplinary care, and biologics, pose challenges.
- Effective management strategies are needed to mitigate complications and improve long-term functional outcomes in s-JIA.
Objectives:
To assess the course, outcome and complications in a mono-centric cohort of 53 patients with systemic onset juvenile idiopathic arthritis (s-JIA).
Methods:
In an observational study, 53 consecutive patients diagnosed with s-JIA on or before October 2009 were enrolled and followed up between October 2009 and September 2012. At each 6-12 weekly visit, clinical examination, laboratory investigations and details of on-going treatment were recorded. Disease course was classified as monocyclic, intermittent and persistent. At last visit, outcome was studied with respect to remission (Wallace criteria) and Steinbrocker functional classification. Juvenile Arthritis Damage Index (JADI) was measured on a subset.
Results:
In 53 patients analysed, the mean follow-up period was 5.5 ± 1.85 y, with a cumulative follow-up period of 291.5 patient-years. The mean age at diagnosis was 6.3 ± 3.4 y. Thirty-three patients suffered from disease and/or drug related complications. Infections were observed in 16 (30%) and macrophage activation syndrome in 5 (9.4%). Nine (17%) had a monocyclic course, 31 (58.5%) had an intermittent course and 13 (24.5%), a persistent course. At last visit, 9/9 patients of the monocyclic group, 17/31 in the intermittent group and 3/13 in the persistent group were in remission. At the end of the study, 96.2% of the index patients were Steinbrocker functional class I and II with the monocyclic group having the best functional outcome. JADI was performed on 20/53 patients. Nine had significant articular damage. The range of Juvenile arthritis damage index-articular (JADI-A) was 0-25/72 (median-6) and of Juvenile arthritis damage index-extra articular (JADI-EA) was 0-4/17 (median-1).
Conclusions:
The outcome of patients with s-JIA in a resource limited setting where early diagnosis, multidisciplinary care and availability of biologics are hurdles, is further altered by complications related to longstanding disease and over use of steroids.
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