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Determing an “autoimmune” phenotype in systemic JIA
Pediatric Rheumatologyvolume 9, Article number: P150 (2011)
Data from research, clinics and response to treatment point out to an autoinflammatory pathogenesis in Systemic onset Juvenile Idiopathic Arthritis (SoJIA). However a subgroup of patients present with c hronic a ctive course of disease, destructive p olyarthritis and production of a utoantibodies (CAPA-subtype) not responsive to anti IL-1 treatment.
Determine phenotypic characteristics of the CAPA-subtype of SoJIA.
Retrospective analysis in a cohort of 35 SoJIA patients well characterized according clinical course and ANA, Rheumatic factor and ANCA.
Autoantibodies have been found in 25,7% of the patients, with ANA being the most frequent auto-AK (table 1). There was a significant difference according the age of onset. Moreover females and carditis have been observed more frequently in the AK-pos. group.
|Observation (month)||mean 69 (range 1-262)|
|percentage for each group|
|onset age (years)||6,03||3,54|
|joints at onset||2,1||3,1|
|joints when AK pos||7|
|SAA||2 of 4||8 of 12|
There is evidence for an autoimmune course of disease in SoJIA in a subset of patients with chronic polyarthritis but without relapses of autoinflammation. These patients probably will need a switch in treatment to prevent further joint destruction.
supported by the BMBF (AID-Net 01GM0907)