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원발성 담즙성 간경변증에 병발된 전신성 홍반성 루푸스의 임상소견을 갖는 쇼그렌 증후군
이장교 ( Chang Gyo Lee ),장현규 ( Hyun Kyu Chang ),김승용 ( Seung Yong Kim ),강호형 ( Ho Hyung Kang ),김덕재 ( Duk Jae Kim ),장진근 ( Chin Kun Chang ),맹주희 ( Ju Hi Maeng ),이희섭 ( Hui Sup Lee ),박배근 ( Pae Gun Park ),정재걸 ( 대한류마티스학회 2001 대한류마티스학회지 Vol.8 No.1
Primary biliary cirrhosis (PBC) is a chronic inflammatory disease characterized by fibrous obliteration of intrahepatic bile ductules. Although the etiopathogenesis of PBC remains unclear, several studies suggest that a disordered immune response may be involved. This disease is commonly associated with various autoimmune diseases, such as Sjogren`s syndrome, scleroderma, rheumatoid arthritis, Hashimoto`s thyroiditis, polymyositis, and pernicious anemia. More than 80% of cases with PBC are accompanied by at least one autoimmune disorder, and 40% by two or more. Sjogren`s syndrome, defined by dry eyes and xerostomia, may be found in 69 to 81% of PBC patients. However, concurrent coexistence of PBC and systemic lupus erythematosus (SLE) has been rarely described in the literature. We report a 46-year-old female with PBC and Sjogren`s syndrome who also satisfied a classification criteria of SLE.