JOINT LESIONS - COMMON EXTRACUTANEOUS MANIFESTATION IN JUVENILE LOCALIZED SCLERODERMA.

Abstract

Aim of the study: to determine the frequency of joint lesions (JnL) in children with juvenile localized scleroderma and it's possible correlation with autoantibodies and markers of fibrosis.

Materials and methods: 500 children with JLS (370 girls and 130 boys) were studied retrospectively for the joint lesion, using standard physical examination, ultrasound examination (UlS) X-ray, MRI. In 190 patients we investigated antinuclear antibodies (antinuclear factor (ANF), rheumatoid factor (RF), antitopoisomerase 1 and anticentomere antibodies, antibodies to DNA, autoantibodies to collagen (Cab) types I-IV, cryoglobulins (CG), serum fibronectine (FN) and hyalyronic acid (HA) levels.

Results: JLS patients were divided into 4 groups:124 patients with circumscribed morphea, 259- linear scleroderma, 93- generalized morphea, pansclerotic in 1 patient, mixed morphea - 23 patients. JnL were noticed in 175 patients (35%), among them the majority 151 patient (86%) with linear and unilateral forms of JLS. JnL were presented by joint pain in 47% of patients, limitation of joint movement in 60% of affected patients, mostly due to periarticular induration or tissue fibrosis. UlS showed joint effusions - in 16% of JnL, sinovitis and tenosinivitis in 45%. In 12 children joint space narrowing was detected by X ray, in 2-articular erosions. MRI was performed in 97 patients with limitation of joint movement, active synovitis, tenosynovitis found in 80 children. In 1 girl with unilateral scleroderma MRI with contrasting visualised avascular osteonecrosis of tibia. The absolute percentage of positive values detected autoantibodies and fibrosis markers was higher in children with JnL. ANF was detected in 56 % and RF in 28,4 % of patients with JnL, while sclerodermo specific antibodies and ds-DNA were detected in small percentage of JnL patients and in none without it. The levels of CG, FN, HA and Cab were elevated in patients with JnL. Cab of type I and type II were detected in most cases of JnL patients (71% and 62% correspondingly).

Conclusion: JnL occurs in 35% of JLS patients, predominantly in linear and unilateral forms of the disease. Detection of autoantibodies and fibrosis markers in 27-56% of JnL cases demonstrates the activity of autoimmune inflammation and justificates early systemic immunosuppressive therapy in JLS.