Ueno Y, Shibata M, Onozuka Y
Department of Internal Medicine, Kawasaki Chuo Hospital.
Nihon Rinsho. 1998 Oct;56(10):2687-98.
Abnormality of humoral and cellular immune functions and the association of autoimmune diseases are frequently observed in primary biliary cirrhosis (PBC). The prevalence of autoimmune diseases was studied in 97 Japanese patients with PBC. Sjögren's syndrome was diagnosed in 33 percent of these patients, arthritis in 22 percent, scleroderma in 11 percent, CREST syndrome in 4 percent, Raynaud's phenomenon in 8 percent, autoimmune thyroiditis in 3 percent, respectively. Fifty-five percent of the patients had at least one autoimmune disease and 19 percent had two or more such disorders. In this study, the prevalence of associated autoimmune diseases was somewhat low compared to that of European and American studies. Geographical variations, however, might exist in the prevalence of autoimmune associations, and the frequent occurrence of coexisting autoimmune diseases suggests an autoimmune pathogenesis in PBC.
在原发性胆汁性肝硬化（PBC）中，经常观察到体液免疫和细胞免疫功能异常以及自身免疫性疾病的关联。对97例日本PBC患者的自身免疫性疾病患病率进行了研究。这些患者中，干燥综合征的诊断率为33%，关节炎为22%，硬皮病为11%，CREST综合征为4%，雷诺现象为8%，自身免疫性甲状腺炎为3%。55%的患者至少患有一种自身免疫性疾病，19%的患者患有两种或更多种此类疾病。在本研究中，与欧美研究相比，相关自身免疫性疾病的患病率略低。然而，自身免疫性关联的患病率可能存在地域差异，自身免疫性疾病的频繁共存提示PBC存在自身免疫发病机制。
