Systemic Diseases Associated with Primary Biliary Cirrhosis: A Report of 10 Cases

The study examined 18 cases of primary biliary cirrhosis (PBC) over 18 years, finding that 10 cases (55.5%) were associated with systemic autoimmune diseases, all in women with an average age of 56.9 years. Commonly associated conditions included Sjögren's syndrome, rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, and MacDuffie syndrome. Treatment with ursodeoxycholic acid showed favorable outcomes in 80% of cases. One patient had urticarial lesions resistant to antihistamines, hypocomplementemic urticarial vasculitis, and hepatic cholestasis, confirmed by liver biopsy. The study highlighted the importance of early detection and treatment of PBC and its associated autoimmune diseases to improve prognosis.