Department of Surgery, Oncology and Gastroenterology-DiSCOG, University of Padova, Via Giustiniani, 2, 35128, Padova, Italy,
Clin Rev Allergy Immunol. 2015 Jun;48(2-3):192-7. doi: 10.1007/s12016-014-8427-x.
There is a paucity of information on extrahepatic autoimmune (EHA) conditions associated with primary biliary cirrhosis (PBC) and on the impact of EHA conditions on PBC patients' survival. Our goal was to assess the association between PBC and other autoimmune diseases and the impact of EHA conditions on the natural history of PBC. We took advantage of 361 consecutive PBC patients enrolled between 1975 and 2012 (22 males, 339 females; mean follow-up 8 ± 6.9 years). Any associated EHA conditions, PBC histological stage at diagnosis, biochemical data, physiological history, and extrahepatic malignancies developing during the follow-up were recorded. Survival was analyzed by means of Kaplan-Meier curves. Importantly, 221 patients (61.2 %) had at least one EHA conditions: 45 patients (20.4 %) had Hashimoto thyroiditis; 7 (3.2 %) had Graves' thyroiditis; 65 (29.4 %) had Raynaud's phenomenon; 124 (56.1 %) had Sjogren's syndrome; 8 (3.6 %) had systemic lupus erythematosus; 22 (9.9 %) had scleroderma; 22 (9.9 %) had rheumatoid arthritis; 18 (8.1 %) had cutaneous autoimmune diseases; 8 (3.6 %) had vasculitis; 5 (1.4 %) had celiac disease; and 25 (13.1 %) had other EHA conditions. The proportion of patients with associated EHA conditions enrolled during representative periods (1975-1980, 1981-1990, 1991-2000, 2001-2010, 2011-2012) remained stable. No differences emerged between patients with versus without EHA conditions in terms of mean age at PBC diagnosis, antimitochondrial antibody (AMA), or antinuclear antibody (ANA) positivity, histological stage at diagnosis, smoking habits, alcohol consumption, or BMI >25. Multiple logistic regression analysis showed that only female gender was significantly associated with positivity for EHA conditions (OR 4.8; 95 % CI 1.6-13.7, p = 0.004). The mean survival after the diagnosis of PBC was much the same in patients with and without EHA conditions. In conclusion, EHA conditions are often associated with PBC, especially in female patients, but they do not reduce patient survival.
原发性胆汁性肝硬化（PBC）患者常合并肝外自身免疫性疾病（EHA），目前有关这些疾病与 PBC 的相关性及对患者预后影响的信息有限。本研究旨在评估 PBC 与其他自身免疫性疾病的相关性，并分析 EHA 对 PBC 自然病程的影响。研究纳入了 1975 年至 2012 年间连续收治的 361 例 PBC 患者（22 例男性，339 例女性；平均随访 8±6.9 年）。记录了患者的任何合并 EHA 疾病、PBC 诊断时的组织学分期、生化数据、生理病史以及随访期间发生的肝外恶性肿瘤。采用 Kaplan-Meier 曲线分析生存情况。重要的是，221 例（61.2%）患者至少有一种 EHA 疾病：45 例（20.4%）患有桥本甲状腺炎；7 例（3.2%）患有格雷夫斯病；65 例（29.4%）患有雷诺现象；124 例（56.1%）患有干燥综合征；8 例（3.6%）患有系统性红斑狼疮；22 例（9.9%）患有硬皮病；22 例（9.9%）患有类风湿关节炎；18 例（8.1%）患有皮肤自身免疫性疾病；8 例（3.6%）患有血管炎；5 例（1.4%）患有乳糜泻；25 例（13.1%）患有其他 EHA 疾病。在不同时期（1975-1980 年、1981-1990 年、1991-2000 年、2001-2010 年、2011-2012 年）纳入的伴有或不伴有 EHA 疾病的患者比例保持稳定。伴有与不伴有 EHA 疾病的患者在 PBC 诊断时的平均年龄、抗线粒体抗体（AMA）或抗核抗体（ANA）阳性率、诊断时的组织学分期、吸烟习惯、饮酒量或 BMI>25 方面均无差异。多因素 logistic 回归分析显示，只有女性是 EHA 疾病阳性的显著相关因素（OR 4.8；95%CI 1.6-13.7，p=0.004）。PBC 诊断后患者的平均生存时间在伴有或不伴有 EHA 疾病的患者中大致相同。总之，EHA 疾病常与 PBC 相关，尤其是在女性患者中，但不会降低患者的生存率。
