IMMUNOLOGICAL FEATURES OF PATIENTS WITH PRIMARY BILIARY-CIRRHOSIS (PBC) OVERLAPPING SYSTEMIC-SCLEROSIS - A COMPARISON WITH PATIENTS WITH PBC ALONE

To characterize the immunological features of patients with primary bi liary cirrhosis (PBC) overlapping systemic sclerosis (SSc), 26 patient s with PBC were classified according to the presence of scleroderma-re lated features (Raynaud's phenomenon, sclerodactyly etc.). The patient s were classified into 10 patients with PBC overlapping SSc (PBC-SSc), four patients with some scleroderma-related features although not mee ting the criteria of SSc (PBC-SSc spectrum) and 12 patients with PBC a lone. Sera from PBC-SSc showed a significantly higher positivity to an ti-centromere antibody (P<0.01) and to E1 beta of pyruvate dehydrogena se complex (P<0.005) than those from patients with PBC alone. The same tendency was observed in PBC-SSc spectrum patients. Patients with PBC exhibiting scleroderma-related features, in both the PBC-SSc and PBC- SSc spectrum, may comprise a subset in PBC, not only clinically, but a lso immunologically.