Takagi T, Kasukawa R
Department of Internal Medicine II, Fukushima Medical College.
Nihon Rinsho. 1994 Jan;52(1):251-5.
Association of autoimmune disease in patients with primary biliary cirrhosis (PBC) was described. Forty-one patients with asymptomatic PBC (a-PBC) and 13 patients with symptomatic PBC (s-PBC) were examined. The overall prevalence of autoimmune disease in association with PBC was 63%. There was no significant difference in the frequency of autoimmune disease association between a-PBC (63.4%) and s-PBC (61.5%). The most frequent complication was Sjögren's syndrome (31.7% in a-PBC and 38.5% in s-PBC). Raynaud's phenomenon was seen in 7 patients (12.2% in a-PBC and 15.4% in s-PBC). Five of these 7 patients showed the CREST syndrome having the anti-centromere antibody. Association of 3 polyarthritis, 2 autoimmune hepatitis, 2 Hashimoto's disease, 1 systemic lupus erythematosus, 1 polymyositis and 1 temporal arteritis was seen in a-PBC patients.
原发性胆汁性肝硬化（PBC）患者自身免疫性疾病的关联情况已被描述。对41例无症状PBC（a - PBC）患者和13例有症状PBC（s - PBC）患者进行了检查。与PBC相关的自身免疫性疾病的总体患病率为63%。a - PBC（63.4%）和s - PBC（61.5%）之间自身免疫性疾病关联的频率无显著差异。最常见的并发症是干燥综合征（a - PBC中为31.7%，s - PBC中为38.5%）。7例患者出现雷诺现象（a - PBC中为12.2%，s - PBC中为15.4%）。这7例患者中有5例表现为伴有抗着丝点抗体的CREST综合征。在a - PBC患者中还发现了3例多关节炎、2例自身免疫性肝炎、2例桥本氏病、1例系统性红斑狼疮、1例多发性肌炎和1例颞动脉炎的关联情况。
