Departament of Internal Medicine, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain.
Departament of Gastroenteroloy and Hepatology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.
Transplant Proc. 2021 Nov;53(9):2695-2697. doi: 10.1016/j.transproceed.2021.06.031. Epub 2021 Sep 28.
Autoimmune liver diseases (ALDs) (primary biliary cholangitis, primary sclerosing cholangitis [PSC], autoimmune hepatitis [AIH]) can present extrahepatic autoimmune manifestations, the most frequent being inflammatory bowel disease (IBD), autoimmune thyroid disease, and Sjögren syndrome (SS).
Retrospective study of patients who have undergone liver transplant (LT) with post-LT follow-up of at least 2 years. Descriptive analysis of clinical variables and overall and graft survival.
ALD was an infrequent indication for LT (68 of 835, 8%), 39 primary biliary cholangitis, 17 AIH, and 12 PSC; 56 were women. The mean (standard deviation [SD]) pre-LT Model for End-Stage Liver Disease score was 17 (5.4). The mean (SD) age of LT recipients at LT was 40 (21) years. A total of 27 patients presented extrahepatic autoimmune diseases. The most frequent was IBD in 7 patients, preferentially in patients with PSC (10/12), followed by Sjögren syndrome and autoimmune thyroid disease. IBD was present in 12 patients: 8 ulcerative colitis (6 PSC and 2 AIH overlap syndrome), 2 Crohn disease both PSC, and another 2 PSC and IBD without conclusive diagnosis (neither for ulcerative colitis nor Crohn disease). Five presented IBD de novo post-LT; the other 7 debuted before LT. In 3 of these 7 patients with pre-LT IBD, the disease went into remission after LT. Colectomy was necessary in 3 patients. No statistically significant findings were found in the survival analysis.
ALD is an infrequent reason for LT. Extrahepatic autoimmune diseases are associated in these patients, with IBD being the most frequent. IBD presents a torpid course but does not impact overall survival.
自身免疫性肝病（ALD）（原发性胆汁性胆管炎、原发性硬化性胆管炎[PSC]、自身免疫性肝炎[AIH]）可表现为肝外自身免疫表现，最常见的是炎症性肠病（IBD）、自身免疫性甲状腺疾病和干燥综合征（SS）。
对接受过肝移植（LT）且 LT 后随访至少 2 年的患者进行回顾性研究。对临床变量、总体和移植物存活率进行描述性分析。
ALD 是 LT 的一个不常见指征（835 例中的 68 例，8%），39 例原发性胆汁性胆管炎，17 例 AIH，12 例 PSC；56 例为女性。LT 前模型终末期肝病评分的平均值（标准差[SD]）为 17（5.4）。LT 时 LT 受者的平均（SD）年龄为 40（21）岁。共有 27 例患者出现肝外自身免疫性疾病。最常见的是 7 例 IBD，主要见于 PSC（12 例中的 10 例），其次是干燥综合征和自身免疫性甲状腺疾病。12 例患者存在 IBD：8 例溃疡性结肠炎（6 例 PSC 和 2 例 AIH 重叠综合征），2 例克罗恩病均为 PSC，另 2 例 PSC 和 IBD 无明确诊断（既不是溃疡性结肠炎也不是克罗恩病）。5 例在 LT 后出现新的 IBD；其余 7 例在 LT 前出现。在这 7 例 LT 前 IBD 患者中，有 3 例疾病在 LT 后缓解。有 3 例患者需要进行结肠切除术。在生存分析中未发现统计学显著差异。
ALD 是 LT 的一个不常见原因。这些患者存在肝外自身免疫性疾病，其中 IBD 最为常见。IBD 呈惰性病程，但不影响总体生存。
