Background Systemic Onset Juvenile Idiopathic Arthritis (SOJIA) is the second most common subtype(23%) of JIA seen in India1. This disease is challenging to diagnose in an environment such as ours where several infectious diseases are endemic. There is sparse literature in this subject from India
To describe the demographic details of children with SOJIA.
To describe the clinical features at disease onset in this cohort.
To study disease patterns & medications used over a median follow up of 36months (mo)
Methods This study is a retrospective review of 49 children with SOJIA followed up over the last 6 mo. Patients were classified as SOJIA according to ILAR criteria. Total of 79 children with this disease have been evaluated since 2009 but 30 children were not included for lack of recent follow up. Data on all patients is systematically collected at disease onset & at every clinic visit. This detailed proforma captures the core set criteria, disease status2 & medications used.
Results Demographic details–A total of 49 children of which 30 were males were included in the data set. Median age of disease onset was 5 yrs(0.58-14yr) with an average delay to diagnosis of 2mo(0-49mo).
Clinical features at disease onset:All patients had fever at onset.82% had fever with arthritis at disease onset but 18% had only fever at onset with median delay to develop arthritis being 2 mo(1-43mo). The commonest joints involved were knees followed by wrists. Six had cervical spine disease, 8 had involvement of small joints of hands, 2 had clinical TMJ involvement & hip disease was noted in 5. Median active joint count at onset was 3 (1-20). At onset, 26 patients had oligoarticular disease, 13 polyarticular & 3 had monoarthritis.
Disease pattern on follow up–The Kaplan Meier(KM) survival curves was used to demonstrate the time taken for patients to reach state of inactivity(IA), CRM(Clinical Remission on Medication) or CROM(Clinical Remission Off Medication). The median time taken for a patient to reach IA disease was 6 months. The KM analysis also demonstrates that around 50% of patients would reach CROM at least once after 5 years of treatment. Median duration of follow up was 30mo(3-114mo).70% children were receiving oral glucocorticoids at 3mo with mean dose(MD) 0.26mg/kg/day(MKD), 17% children at end of 1 year (MD 0.24 MKD) & 5% at end of 3 years (MD 0.16 MKD).78% children required methotrexate(MTX) at 3mo, all at 1 year & 90% at 3yrs. Thalidomide was given to 4% within 3 mo. of onset, 14% at 1yr & 16% at 3 years.6% patients received biologics within 3 mo. of treatment onset(33% Tocilizumab TCZ & 67% Etanercept).7% were receiving TCZ at the end of 1yr & 5% at the end of 3yrs.
Conclusions SOJIA is seen at younger age with more boys in India. Majority had oligoarticular disease. All patients needed steroids & Methotrexate. 22% patients required biologics. Macrophage Activation Syndrome was seen in 12, 6 patients at onset & 6 in later course of disease. At 3 yr follow up, 6 had active disease, 3 had IA disease, 8 had CRM & 2 patients had CROM. There was no mortality.
Agarwal M et al. Outcome Of 267 JIA Patients From A Tertiary Level Indian Pediatric Rheumatology Centre. Ann Rheum Dis 2012;71(Suppl3):433.
Wallace CA et al. Preliminary criteria for clinical remission for select categories of juvenile idiopathic arthritis. J Rheumatol. 2004;31(11):2290
Disclosure of Interest None Declared