MUSCULOSKELETAL DISORDERS IN JUVENILE-ONSET MIXED CONNECTIVE-TISSUE DISEASE

Objective. We studied the occurrence of musculoskeletal involvement in patients with juvenile onset mixed connective tissue disease (MCTD) a nd their functional ability. Methods, Thirteen patients who fulfilled the Kasukawa criteria of MCTD, were subjected to a general orthopedic assessment, as well as to a specific examination of tenderness and swe lling of joints, muscle strength, joint motion and alignment, radiogra phical evaluation, and on disability and discomfort. Results, Low indi ces were found on the modified Ritchie Articular Index (RI) (0.07) and the Fuchs Swelling Index (0.13), Mild to moderate indices were measur ed on the Joint Alignment and Motion Scale (JAM) (0.52) and on the Lar sen Radiographic Evaluation Scales for the wrist, metacarpal, proximal and distal interphalangeal joints (L-RES) (1.40, 0.62, 0.31, 0.31). T he mean pulp-thickness ratio was 22%, which is an indication for scler odermic changes in the hands. Proximal muscle weakness was found in 10 /13 patients (mainly in the upper extremity), distal muscle weakness w as found in 12/13 patients. On the pediatric version of the Stanford H ealth Assessment Questionnaire (C-HAQ), pain and discomfort scored 0.0 4, disability scored 0.46; those scores were considered to be low, Bet ween L-RES and JAM a significant correlation was found (0.74, 0.74 and 0.73; p <0.01). Another significant correlation was found between the RI and the disablity score of the HAQ (0.58; p <0.05). Conclusion. Sc lerodermic changes as well as arthritic changes are of a benign charac ter in our population of patients with juvenile onset MCTD.