[Circumscribed scleroderma: internal manifestations and significant correlation to HLA-DR1 and DR5]

Abstract

In 44 patients with morphea the incidence of internal organ involvement was studied. For the clinical study, only patients with disseminated (22 patients), linear (20 patients), and generalized morphea (2 patients) were considered. Systemic parameters were determined for inflammation and the function of the esophagus, lung, heart and kidneys. In 22 patients, mostly with the linear form of morphea, the muscles were studied by electromyography. In 23 patients the HLA-A, HLA-B, HLA-C and HLA-DR patterns were determined; 27% of the patients showed systemic organ manifestations. Esophagus function was impaired in 10 and lung function in 6 cases; 15 patients showed myositis. The degree of systemic involvement was correlated with the type of morphea and the grade of systemic inflammation. Generalized morphea showed a high rate of organ involvement (2 of 2); in linear morphea organ involvement was reduced to 34% and in the disseminated form, to 14%. There was a significant association of HLA-DR1 and -DR5 with the different types of morphea.