Rodriguez Jose A, Bang Tami J, Restrepo Carlos S, Green Daniel B, Browne Lorna P, Vargas Daniel
Faculty of Medical Sciences, Universidad Nacional Autónoma de Honduras, Tegucigalpa, Honduras (J.A.R.); Department of Radiology, Division of Cardiopulmonary Imaging, University of Colorado School of Medicine, 12401 E 17th Ave, Aurora, CO 80045 (T.J.B., D.V.); Department of Radiology, University of Texas Health Science Center, San Antonio, Tex (C.S.R.); Department of Radiology, Weill Cornell Medicine, New York, NY (D.B.G.); and Department of Radiology, Children's Hospital Colorado, Aurora, Colo (L.P.B.).
Radiol Cardiothorac Imaging. 2021 Mar 25;3(2):e200418. doi: 10.1148/ryct.2021200418. eCollection 2021 Apr.
Primary immunodeficiency disorders (PIDs), which are humoral, combined, and innate defects of the immune system, are relatively uncommon and may go undiagnosed in patients experiencing recurrent infections, resulting in increased morbidity and mortality. PIDs are clinically characterized by a broad spectrum of disorders, including repeated infections, autoimmune disorders, lymphoproliferative diseases, congenital anomalies, and increased risk of malignancy. Cardiothoracic imaging plays a crucial role in the diagnosis of PIDs owing to the high rates of repeated respiratory infections leading to bronchiectasis and other forms of chronic lung disease. Although PIDs as a group may seem similar in terms of radiologic features and clinical manifestations, there are specific entities that are pertinent to each PID on an individual level. For example, patients with common variable immunodeficiency may develop a unique granulomatous lymphocytic interstitial lung disease, and Good syndrome is associated with thymoma. Familiarity with the imaging characteristics of these disorders may expedite diagnosis and prognostication, and better direct therapy. Reviewing the thoracic manifestations of all PIDs is beyond the scope of this article; thus, the focus herein is on discussing the thoracic manifestations of the most common PIDs and their imaging features. © RSNA, 2021An earlier incorrect version appeared online. This article was corrected on March 25, 2021.
原发性免疫缺陷病（PIDs）是免疫系统的体液、联合和先天性缺陷，相对不常见，在反复感染的患者中可能未被诊断出来，从而导致发病率和死亡率增加。PIDs的临床特征是一系列广泛的疾病，包括反复感染、自身免疫性疾病、淋巴增殖性疾病、先天性异常以及恶性肿瘤风险增加。由于反复呼吸道感染导致支气管扩张和其他形式的慢性肺病的发生率很高，心胸成像在PIDs的诊断中起着至关重要的作用。尽管作为一个整体，PIDs在放射学特征和临床表现方面可能看起来相似，但在个体层面上，每种PIDs都有特定的相关实体。例如，常见变异型免疫缺陷患者可能会发展出一种独特的肉芽肿性淋巴细胞间质性肺病，而古德综合征与胸腺瘤有关。熟悉这些疾病的影像学特征可能会加快诊断和预后评估，并更好地指导治疗。回顾所有PIDs的胸部表现超出了本文的范围；因此，本文的重点是讨论最常见PIDs的胸部表现及其影像学特征。© RSNA，2021 早期的错误版本已在线发布。本文于2021年3月25日进行了更正。
