Background The ERA subcategory forms a major proportion of children with JIA in India (36%) (1) whereas western literature suggests ERA in JIA patients to be 6-19% (2,3). In these patients, inflammatory back pain has been suggested to occur infrequently at onset. This is not our experience. This study was taken up to systematically address these questions.
Objectives 1.) To study the demographic and clinical profile of patients with ERA 2.) To compare it to the western data available
Methods All data was prospectively collected on standardised proformas for all ERA patients from 2009 August to January 2012.
Results Demographics: A total of 110 patients with ERA were seen through this period. Of these 88% (96) were boys with a median age of onset 10.79 years (range 1.63-15.83).10.9% had a positive family history of spondyloarthropathy.
Clinical Features at onset (as defined by clinical features seen in the first 6 months of clinical disease)Peripheral joint disease seen in 87.3%, axial disease in 46.3%, enthesitis in 48.2%. Of all patients with axial disease, 4.5% had only cervical spine involvement without sacroiliitis. Of the 12.7%patients who had no peripheral joint disease; 9% had only axial disease and 3.6% had axial disease with enthesitis. Hip involvement, a poor prognostic sign, was seen in 23%.The median no. of active joint count was 3 with a strikingly high no. of patients who had a tarsus/metatarsal involvement at 33.6%. Of this cohort 95.4% were HLA B 27 positive. Uveitis was seen in 21.8%, of which 87.5% had acute anterior uveitis and 12.5% persisted with chronic uveitis.
Comparison with western data: Only one publication by Pagnini et al from Europe described features of ERA at onset (4). In this study, of the 59 patients, 30% developed sacroiliitis a year after onset, 58% had mid foot involvement, 66% were HLA B 27 positive and this cohort comprised only 28% of the total no. of JIA patients at that centre.
Conclusions This study, the first from India describes a large cohort of ERA patients who differ significantly from their western counterparts. The patients from our centre have higher proportion of HLA B 27 positivity, early onset axial and midfoot disease with paucity of positive family history. This study needs to be replicated from other centres in India.
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Demirkaya E, Ozen S, Bilginer Y, Ayaz NA, Makay BB, Unsal E, et al. The distribution of juvenile idiopathic arthritis in the eastern Mediterranean: results from the registry of the Turkish Paediatric Rheumatology Association. Clinical and experimental rheumatology;29 1):111–116.
Thompson SD, Barnes MG, Griffin T a, Grom A a, Glass DN. Heterogeneity in juvenile idiopathic arthritis: impact of molecular profiling based on DNA polymorphism and gene expression patterns. Arthritis and rheumatism.2010 Sep;62(9):2611–2615.
Pagnini I, Savelli S, Matucci-Cerinic M, Fonda C, Cimaz R, Simonini G. Early predictors of juvenile sacroiliitis in enthesitis-related arthritis. The Journal of rheumatology. 2010 Nov; 37(11):2395-2401.
Disclosure of Interest None Declared