Autoantibodies associated with primary biliary cholangitis are common among patients with systemic lupus erythematosus even in the absence of elevated liver enzymes.,Clinical & Experimental Immunology

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Autoantibodies associated with primary biliary cholangitis are common among patients with systemic lupus erythematosus even in the absence of elevated liver enzymes.
Clinical & Experimental Immunology ( IF 3.4 ) Pub Date : 2020-09-10 , DOI: 10.1111/cei.13512
A Ahmad ₁ , R Heijke ₂ , P Eriksson ₂ , L Wirestam ₂ , S Kechagias ₃ , C Dahle ₁ , C Sjöwall ₂

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Knowledge of concomitant autoimmune liver diseases (AILD) is more detailed in primary Sjögren’s syndrome (pSS) compared to systemic lupus erythematosus (SLE). Herein, the prevalence of autoantibodies associated with autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) was investigated in stored sera from patients with SLE (n = 280) and pSS (n = 114). Antibodies against mitochondria (AMA), liver–kidney microsomal (LKM) antigen, smooth muscle (SMA) and anti‐nuclear antibodies (ANA) were analysed with immunofluorescence microscopy. In addition, AILD‐associated autoantibodies were tested with immunoblot. Prior to sampling, eight SLE (2·9%) and three pSS (2·6%) cases were diagnosed with AILD. Among SLE‐cases without known AILD (n = 272), 26 (9·6%) had PBC‐associated autoantibodies, 15 (5·5%) AIH‐associated autoantibodies (excluding ANA) and one serological overlap. Most subjects with PBC‐associated autoantibodies had liver enzymes within reference limits (22 of 27, 81%) or mild laboratory cholestasis (two of 27, 7·4%), while one fulfilled the diagnostic PBC‐criteria. AMA‐M2 detected by immunoblot was the most common PBC‐associated autoantibody in SLE (20 of 272, 7·4%). The prevalence of SMA (4·4%) was comparable with a healthy reference population, but associated with elevated liver enzymes in four of 12 (25%), none meeting AIH‐criteria. The patient with combined AIH/PBC‐serology had liver enzymes within reference limits. Among pSS cases without known AILD (n = 111), nine (8·1%) had PBC‐associated, 12 (10·8%) AIH‐associated autoantibodies and two overlapped. PBC‐associated autoantibodies were found as frequently in SLE as in pSS but were, with few exceptions, not associated with laboratory signs of liver disease. Overall, AILD‐associated autoantibodies were predominantly detected by immunoblot and no significant difference in liver enzymes was found between AILD autoantibody‐negative and ‐positive patients.

中文翻译：

即使肝酶不升高，系统性红斑狼疮患者中与原发性胆汁性胆管炎相关的自身抗体也很常见。

与系统性红斑狼疮 (SLE) 相比，对原发性干燥综合征 (pSS) 伴随的自身免疫性肝病 (AILD) 的了解更为详细。在此，我们对 SLE ( n = 280) 和 pSS ( n = 114)患者的储存血清中与自身免疫性肝炎 (AIH) 和原发性胆汁性胆管炎 (PBC) 相关的自身抗体的患病率进行了调查。使用免疫荧光显微镜分析抗线粒体（AMA）、肝肾微粒体（LKM）抗原、平滑肌（SMA）和抗核抗体（ANA）的抗体。此外，还使用免疫印迹测试了 AILD 相关自身抗体。采样前，8 例 SLE (2·9%) 和 3 例 pSS (2·6%) 病例被诊断为 AILD。在没有已知 AILD 的 SLE 病例中（n = 272），26 例（9·6%）有 PBC 相关自身抗体，15 例（5·5%）有 AIH 相关自身抗体（不包括 ANA），1 例血清学重叠。大多数携带 PBC 相关自身抗体的受试者的肝酶均在参考限值内（27 人中的 22 人，81%）或轻度实验室胆汁淤积（27 人中的 2 人，7·4%），而其中一名受试者满足 PBC 诊断标准。通过免疫印迹检测到的 AMA-M2 是 SLE 中最常见的 PBC 相关自身抗体（272 例中的 20 例，7·4%）。SMA 的患病率 (4·4%) 与健康参考人群相当，但 12 人中有 4 人 (25%) 与肝酶升高相关，但没有人符合 AIH 标准。合并 AIH/PBC 血清学检查的患者的肝酶水平在参考范围内。在无已知 AILD 的 pSS 病例中 ( n = 111)，9 例 (8·1%) 具有 PBC 相关自身抗体，12 例 (10·8%) 具有 AIH 相关自身抗体，2 例存在重叠。PBC 相关自身抗体在 SLE 中与 pSS 中一样常见，但除少数例外，与肝病的实验室症状无关。总体而言，AILD 相关自身抗体主要通过免疫印迹检测，并且 AILD 自身抗体阴性和阳性患者之间的肝酶没有发现显着差异。

更新日期：2020-09-10

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