JOINT LESIONS - COMMON EXTRACUTANEOUS MANIFESTATION IN JUVENILE LOCALIZED SCLERODERMA

Area of Science:

• Pediatric Rheumatology
• Dermatology
• Immunology

Background:

• Juvenile localized scleroderma (JLS) is a chronic autoimmune condition affecting skin and connective tissues.
• Joint involvement (JnL) in JLS can lead to significant morbidity.
• Understanding the prevalence and associations of JnL is crucial for effective management.

Purpose of the Study:

• To determine the frequency of joint lesions (JnL) in children diagnosed with juvenile localized scleroderma (JLS).
• To investigate the potential correlation between JnL and the presence of specific autoantibodies and markers of fibrosis in JLS patients.

Main Methods:

• Retrospective analysis of 500 children with JLS, assessing joint lesions via physical examination, ultrasound, X-ray, and MRI.
• Investigation of autoantibodies including antinuclear factor (ANF), rheumatoid factor (RF), antitopoisomerase 1, anticentromere antibodies, anti-dsDNA, and anti-collagen antibodies (Cab).
• Measurement of fibrosis markers such as cryoglobulins (CG), fibronectin (FN), and hyaluronic acid (HA) in 190 patients.

Main Results:

• Joint lesions (JnL) were identified in 35% of JLS patients, predominantly in linear (86%) and unilateral forms.
• JnL manifested as joint pain (47%) and limited movement (60%), often due to fibrosis. Ultrasound revealed synovitis in 45% of cases.
• Elevated levels of autoantibodies (ANF in 56%, RF in 28.4%) and fibrosis markers (CG, FN, HA, Cab) were observed more frequently in patients with JnL.

Conclusions:

• Joint lesions are a common complication in JLS, occurring in 35% of cases, with a predilection for linear and unilateral presentations.
• The increased detection of autoantibodies and fibrosis markers in children with JnL suggests ongoing autoimmune activity.
• These findings support the consideration of early systemic immunosuppressive therapy in JLS patients presenting with joint involvement.