Abstract

Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The incidence rate among Caucasians is reported to be 8-15/100 000/year (1, 2). The long-term prognosis is insufficiently studied in JIA, although it is known that the disease often proceeds into adulthood and may cause joint damage leading to significant morbidity and physical disability. There are many challenges in elucidating the long-term prognosis. Amongst others, frequent occurrence of disease misclassification in diagnostic registers and the heterogeneity of JIA have made it difficult to interpret results (3, 4). Objectives To study the epidemiology, incidence and long-term outcome of JIA in southern Sweden using a population-based cohort of children with a validated diagnosis of JIA collected over nine years. Methods Potential cases of JIA, diagnosed between 2002 and 2010 were collected from a local search at the Department of Rheumatology in Lund and at the National Board for Health and Welfare, using the ICD-codes M08-M09. The study area is Skane, the southernmost county of Sweden (population 1.24 million; 19.1% aged Results 251 cases of JIA were confirmed. The mean annual incidence rate for JIA was estimated to be 12.8/100 000/year. The highest age specific annual incidence is at the age of two years (36/100 000/year). This peak is consistent in the female group, but the incidence peak among males is at twelve years of age. Almost all patients are at some point during their disease course prescribed non-steroidal anti-inflammatory drugs (98%). Intra-articular steroid injections are also frequently used (78.9% in the total cohort). Methotrexate is the most common disease modifying anti-rheumatic drug prescribed (60.6%). Tumor necrosis factor alpha-inhibitors are used as treatment in 23.9% of the children. Oligoarthritis was the largest subgroup (44.7%), followed by undifferentiated JIA (16.3%), polyarticular rheumatoid factor negative JIA (13.9%), enthesitis-related arthritis (8.8%), polyarticular rheumatoid factor positive JIA (6.8%), juvenile psoriatic arthritis (6.8%) and systemic JIA as the smallest subgroup (2.8%). Uveitis, both acute and chronic, was seen in 10.8% of the children. Permanent joint affection was seen in 20.7% of the children and 8.8% have been treated with joint corrective orthopedic surgery. At five-year follow-up, 57% of the children had disease activity, defined as arthritis and/or uveitis (2.1% with uveitis as active disease). Conclusion This is a well-defined, population-based and validated cohort of children diagnosed with JIA investigating long-term outcome in the era of biologics. We found that a considerable part of the children still develop uveitis, permanent joint affection and need joint corrective surgery. Surprisingly, more than 50% of the cohort has active disease five years after diagnosis. In conclusion, we still have long-term challenges in children with JIA, in spite of state-of-the-art treatment.

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