Akulkina L A, Brovko M Yu, Sholomova V I, Rozina T P, Yanakayeva A S, Frantsuzevich L Ya, Lebedeva M V, Fomin V V
M.V. Lomonosov Moscow State University, Faculty of Base Medicine, Department of Internal medicine.
I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Tareev clinic of Internal Diseases.
Ter Arkh. 2018 Aug 27;90(8):107-112. doi: 10.26442/terarkh2018908107-112.
The primary autoimmune liver diseases conventionally include primary biliary cholangitis, primary sclerosing cholangitis and autoimmune hepatitis. Despite of primary autoimmune affection of different parts of the hepatobiliary system, in the recent decades, a lot of data has emerged indicating the presence of extrahepatic manifestations of these diseases, in particular, lung lesions, such as nodular and interstitial changes with possible progression and development of fibrosis and respiratory failure. In case of lungs disease, both pulmonary parenchyma and lung vessels, pleura, and intrathoracic lymph nodes can be involved. The most sensitive and specific procedure to assess the extent of the lung lesions and their evolution is high-resolution computed tomography. Due to the possibility of long-term asymptomatic course of the pulmonary disease with development of irreversible changes in patients with autoimmune liver diseases, it seems reasonable to conduct screening studies aimed at early detection and treatment of lung lesions in this population.
传统上，原发性自身免疫性肝病包括原发性胆汁性胆管炎、原发性硬化性胆管炎和自身免疫性肝炎。尽管这些疾病主要是对肝胆系统不同部位的自身免疫性损害，但近几十年来，大量数据表明这些疾病存在肝外表现，特别是肺部病变，如结节性和间质性改变，并可能进展为纤维化和呼吸衰竭。在肺部疾病中，肺实质、肺血管、胸膜和胸内淋巴结均可受累。评估肺部病变范围及其演变的最敏感和特异的检查方法是高分辨率计算机断层扫描。由于自身免疫性肝病患者的肺部疾病可能长期无症状进展并出现不可逆变化，因此对该人群进行旨在早期发现和治疗肺部病变的筛查研究似乎是合理的。
