CLINICAL MANIFESTATIONS AND IMMUNOLOGICAL FEATURES OF PRIMARY SJOGRENS-SYNDROME WITH LIVER INVOLVEMENT - ANALYSIS OF 30 CASES

Objective To evaluate the incidence, severity, clinical manifestations and immunological features relevant to liver involvement in 135 cases of primary Sjogren's syndrome. Methods One hundred and thirty-five pa tients with definite primary Sjogren's syndrome were analyzed retrospe ctively for liver involvement by the abnormalities of the liver enzyme s, bilirubin level and liver biopsied section. Results The liver invol vement in 30 patients (22.2%) could be etiologically ascribed to Sjogr en's syndrome itself. The clinical spectrum and severity of this entit y differed widely, 36.6% showed no relevant clinical symptoms, however jaundice was found in 46.7% of patients. Six patients showed patholog ical changes of chronic active hepatitis. 73.3% of all patients with l iver involvement responded to steroid and immunosuppressive drugs, yet with a tendency to relapse(two cases). Liver cirrhosis was developed in five cases. The spectrum of serum autoantibodies in the patients wi th liver involvement showed no difference from those without liver inv olvement. Most of them were compatible with the serum profile of autoi mmune hepatitis type-1. Conclusions Liver involvement was complicated in 22.2% patients of primary Sjogren's syndrome. Clinical manifestatio ns were non-specific and the main pathological change was chronic acti ve hepatitis. The differential diagnosis between Sjogren's syndrome wi th liver involvement and type-1 autoimmune hepatitis could be only asc ribed to other systemic clinical manifestations of Sjogren's syndrome.