OBSOLETE: Rheumatoid factor-negative juvenile idiopathic arthritis with anti-nuclear antibodies

Rheumatoid factor-negative juvenile idiopathic arthritis with anti-nuclear antibodies (ANA-positive RF-negative JIA) was a proposed subclassification of juvenile idiopathic arthritis (JIA) that has since been marked as obsolete in the Orphanet classification system. This entity referred to children who developed chronic arthritis before the age of 16, tested negative for rheumatoid factor (RF), but were positive for anti-nuclear antibodies (ANA). The condition primarily affects the musculoskeletal system, causing joint inflammation, pain, swelling, and stiffness, particularly in the large and small joints. ANA positivity in JIA is clinically significant because it is associated with an increased risk of chronic anterior uveitis (inflammation of the eye), which can lead to vision loss if not detected and treated early. The condition predominantly affects young girls. As an obsolete classification, this entity has been largely absorbed into broader or revised JIA categories. Current classification efforts, including proposals from the Pediatric Rheumatology International Trials Organisation (PRINTO), have moved toward recognizing ANA-positive JIA as a distinct homogeneous entity regardless of the number of joints involved. Treatment for the underlying arthritis typically includes nonsteroidal anti-inflammatory drugs (NSAIDs), intra-articular corticosteroid injections, and disease-modifying antirheumatic drugs (DMARDs) such as methotrexate. Biologic therapies, particularly TNF inhibitors and IL-6 receptor antagonists, are used in refractory cases. Regular ophthalmologic screening is essential due to the elevated uveitis risk associated with ANA positivity.

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