Karakaya Fatih, Oztekin Sura, Ozturk Yelda, Kalkan Cagdas, Melekoglu Ellik Zeynep, Halil Elhan Atilla, Soykan Irfan, Idilman Ramazan
Department of Gastroenterology, Ankara University School of Medicine, Ankara, Turkey.
Department of Internal Medicine, Ankara University School of Medicine, Ankara, Turkey.
Hepatol Forum. 2021 Jan 8;2(1):3-6. doi: 10.14744/hf.2020.2020.0028. eCollection 2021 Jan.
This study aimed to determine the presence of concomitant extrahepatic autoimmune disease (EAD) in patients with autoimmune liver disease (ALD) and the efficacy of the treatment response of ALD with the presence of any EAD.
Between January 2001 and November 2017, 241 patients with ALD were included in the study.
Of the 241 patients, 88, 134, and 19 had autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and overlap syndrome (OS), respectively. Thirty-one patients had cirrhosis: 77% and 23% had compensated and decompensated disease, respectively. The presence of at least one EAD was defined in 38.6% of the patients with ALD (n=93), and 12% of them had ≥1 EAD. EAD was most commonly seen in patients with OS and PBC compared with those with AIH (p=0.036). Autoimmune thyroid disease was the most common association (20%), followed by Sjogren syndrome (12.0%). At the end of the follow-up period, 165 patients (72%) had biochemical response. The presence of EAD did not affect the biochemical response.
EAD is most frequently seen in patients with ALD. The presence of EAD is not associated with the treatment response.
本研究旨在确定自身免疫性肝病（ALD）患者中是否存在合并的肝外自身免疫性疾病（EAD），以及存在任何EAD时ALD的治疗反应疗效。
2001年1月至2017年11月期间，241例ALD患者纳入本研究。
241例患者中，分别有88例、134例和19例患有自身免疫性肝炎（AIH）、原发性胆汁性胆管炎（PBC）和重叠综合征（OS）。31例患者患有肝硬化：分别有77%和23%的患者患有代偿期和失代偿期疾病。38.6%的ALD患者（n = 93）被定义为存在至少一种EAD，其中12%的患者患有≥1种EAD。与AIH患者相比，EAD在OS和PBC患者中最常见（p = 0.036）。自身免疫性甲状腺疾病是最常见的关联疾病（20%），其次是干燥综合征（12.0%）。随访期结束时，165例患者（72%）有生化反应。EAD的存在不影响生化反应。
EAD在ALD患者中最常见。EAD的存在与治疗反应无关。
