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.