Zukowski T H, Jorgensen R A, Dickson E R, Lindor K D
Mayo Foundation, Rochester, Minnesota, USA.
Am J Gastroenterol. 1998 Jun;93(6):958-61. doi: 10.1111/j.1572-0241.1998.00287.x.
A variety of autoimmune conditions occur in association with primary biliary cirrhosis. Among these conditions are sicca syndrome, Raynaud's phenomenon, arthritis, and Hashimoto's thyroiditis. Information is sparse regarding the prevalence and natural history of these conditions when associated with primary biliary cirrhosis and their response to ursodeoxycholic acid treatment. We evaluated the prevalence, natural history, and response to ursodeoxycholic acid therapy of these conditions coassociated with primary biliary cirrhosis.
One hundred-eighty patients with primary biliary cirrhosis, enrolled in a prospective randomized controlled trial of ursodeoxycholic acid (13-15 mg/ kg/day), were included. Patients were assessed at study entry and annually.
At entry, 77/180 patients (43%) had one of the four conditions, and 18/180 patients (10%) had two or more conditions. Sicca syndrome was the most common, occurring in 58/180 patients (32%). After 2 yr, there was no difference between the treatment groups with regard to resolution or spontaneous onset of these autoimmune features. Sicca syndrome was the most common spontaneously developing condition (9% per yr). Sicca syndrome was the most common associated autoimmune condition, present in one-third of our patients. The associated conditions tended to improve over time, with a low rate of spontaneously developing these conditions. Although ursodeoxycholic acid therapy leads to improvement in the underlying liver disease, it did not appear to influence either the development or resolution of these autoimmune features.
Although ursodeoxycholic acid is beneficial in the treatment of primary biliary cirrhosis, it had no measurable effect on the autoimmune conditions coassociated with the disease.
多种自身免疫性疾病与原发性胆汁性肝硬化相关。这些疾病包括干燥综合征、雷诺现象、关节炎和桥本甲状腺炎。关于这些疾病与原发性胆汁性肝硬化相关时的患病率、自然史及其对熊去氧胆酸治疗的反应，信息较少。我们评估了与原发性胆汁性肝硬化相关的这些疾病的患病率、自然史以及对熊去氧胆酸治疗的反应。
纳入180例原发性胆汁性肝硬化患者，这些患者参加了一项熊去氧胆酸（13 - 15 mg/kg/天）的前瞻性随机对照试验。在研究开始时和每年对患者进行评估。
入组时，77/180例患者（43%）患有这四种疾病中的一种，18/180例患者（10%）患有两种或更多种疾病。干燥综合征最为常见，发生于58/180例患者（32%）。2年后，治疗组在这些自身免疫特征的缓解或自发出现方面没有差异。干燥综合征是最常见的自发出现的疾病（每年9%）。干燥综合征是最常见的相关自身免疫性疾病，在我们三分之一的患者中存在。随着时间推移，相关疾病往往有所改善，这些疾病的自发发生率较低。虽然熊去氧胆酸治疗可改善潜在的肝脏疾病，但它似乎并未影响这些自身免疫特征的发生或缓解。
虽然熊去氧胆酸对原发性胆汁性肝硬化的治疗有益，但它对与该疾病相关的自身免疫性疾病没有可测量的影响。
