ABSTRACT
BACKGROUND: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology. The disease is relatively common in western countries, but so far, only about 20 cases have been reported in Korea. Thus, the purpose of our prospective study was to evaluate the clinical characteristics of PBC in Korea. METHODS: Between October 1994 and February 1999, 16 patients diagnosed as PBC at our department were enrolled in this study. We analyzed these patients for the distribution of age and sex, initial symptoms and signs, associated disorders, laboratory, endoscopic, and radiologic and histologic findings. RESULTS: Of the 16 patients, the ratio of male to female was 1:7 and the average age was 57.5 years. Pruritus (37.5%) was the most frequent presenting symptom followed by xerostomia and xerophthalmia (12.5%), jaundice (6.3%), chronic fatigue (6.3%), melena (6.3%). Associated disorders were Sj gren's syndrome (25%), arthropathy (12.5%), Raynaud's phenomenon (6.3%) and diabetes (6.3%). Abnormalities of liver function were found frequently. Antimitochondrial antibody (100%), antinuclear antibody (31.25%) and rheumatoid factor (31.25%) were found. Long-term administration of UDCA improved both clinical and biochemical signs in most patients, however, 2 patients experienced exacerbation during UDCA therapy. CONCLUSIONS: We experienced 16 cases of PBC within a short period. It is expected that we can find these patients more frequently if we suspect this disease in patients with pruritus and unexplained obstructive pattern of liver function abnormality.