Prognosis, complications and treatment response in systemic juvenile idiopathic arthritis patients: A single‐center experience

Abstract

Aim Systemic juvenile idiopathic arthritis (sJIA) is a distinctive subtype of JIA characterized by systemic features and poor outcome. We aimed to investigate demographic and clinical features, long‐term treatment response and disease complications in a large sJIA cohort. Methods Patients diagnosed with sJIA followed up at a pediatric rheumatology outpatient department from January 2003 to December 2017 were included. Demographic and clinical features, long‐term treatment response and disease complications were retrospectively collected. Results A total of 168 sJIA patients (51.8% female, 48.2% male) were included: 31.5% with monocyclic, 13.7% polycyclic and 54.8% with persistent clinical course. Corticosteroids were initially used in all patients. Methotrexate was used in 75% and cyclosporine A was used in 17.3% patients. Biological drugs were used in 42.8% patients; etanercept in 29.7%, anakinra in 16%, canakinumab in 16%, tocilizumab in 10% patients. Remission off medication was achieved in 82 (48.8%). Macrophage activation syndrome (MAS) was present in 11.9%, growth retardation in 11.3% patients. Eight percent (4/50) of patients had low bone mineral density. Three patients (1.78%) died due to MAS secondary multiorgan insufficiency and infection. Conclusion The disease is characterized with diverse clinical presentation and possibly severe complications. MAS complicated with multiorgan insufficiency is the major mortality factor. Corticosteroids represent the mainstay of the initial treatment. In patients resistant to classic treatment, biological drugs should be timely introduced.

Systemic inflammation and chronic kidney disease in a patient due to the RNASEH2B defect

“…Systemic juvenile idiopathic arthritis (SoJIA), and later onset chronic infantile neurologic, cutaneous, and arthritis (CINCA) syndrome were once suspected. Different from the clinical manifestations of this patient, chilblains and intracranial calci cation are not present in SoJIA or CINCA; leukocytosis, destructive arthritis, or macrophage activation syndrome (MAS) are noted in SoJIA [4][5][6]; visual impairment, sensor neural deafness or progressive chronic meningitis have been commonly reported in CINCA [5]. Chronic kidney disease due to amyloidosis has been rarely reported in SoJIA, which is common in CINCA (Table 2) [8].…”

Juvenile Idiopathic Arthritis in South Australia

“…In a recent systemic JIA cohort study in Turkey [27], the frequencies of growth retardation and MAS were 11.3% (n = 19/168) and 11.9% (n = 20/168), respectively. In the German Biologics JIA Registry (BIKeR), 4.5% of their systemic JIA patients experienced MAS [53].…”

“…Complications and comorbidities were recorded and categorised based on case-note reports. Complications categories included: ocular, musculoskeletal, medication intolerance and side effects, growth failure/retardation, amyloidosis and macrophage activation syndrome [27][28][29][30][31][32]. Ocular complications included uveitis and uveitis-related complications such as glaucoma, cataract and macular oedema [29,33].…”

Growth parameters of Turkish children with juvenile idiopathic arthritis

“…Previous publications showed that growth of JIA patients affected by disease duration, disease activity, age at the disease onset, dose and length of corticosteroid usage, JIA subgroup with different results [13][14][15][16][17][18][19][20] . Moreover, previous reports have suggested that anti-TNF treatments, particularly etanercept have significantly positive effect on growth of JIA patients 21,22 .…”