我们这篇综述的目的是让放射科医生熟悉在原发性免疫缺陷疾病成人患者中观察到的肺部并发症的初始和进展性 CT 表现，包括原发性抗体缺乏 (PAD)、高 IgE 综合征 (HIES) 和慢性肉芽肿病（CGD）。在 PAD 患者中，反复肺部感染可能导致气道重塑，导致支气管壁增厚、支气管扩张、粘液堵塞、马赛克灌注和呼气滞留。间质性肺疾病与肺淋巴样增生、肉芽肿性炎症和机化性肺炎相关，称为肉芽肿-淋巴细胞性间质性肺疾病（GLILD）。 GLILD 的 CT 特征是实性和半实性肺部结节以及气腔实变区域、网状混浊和淋巴结肿大。这些特征可能与粘膜相关淋巴组织 (MALT) 淋巴瘤的特征重叠，从而证明活检的合理性。在 HIES 患者中，尤其是常染色体显性遗传型（乔布综合征）患者，反复的化脓性感染会导致永久性肺损伤。在先前存在的气肿和支气管扩张区域发生曲霉菌的继发感染，导致慢性气道感染。 CT 肺曲霉病的完整谱系可见，包括曲霉瘤、慢性空洞性肺曲霉病、过敏性支气管肺曲霉病 (ABPA) 样型、混合型和侵袭性型。 CGD 患者反复出现细菌和真菌感染，导致实质疤痕、牵拉性支气管扩张、瘢痕性肺气肿、气道重塑和嵌合体。侵袭性曲霉菌病是死亡的主要原因，表现为单个或多个结节、空腔实变区域，可能并发脓肿、脓胸或连续延伸至胸膜或胸壁。临床相关性声明：了解原发性免疫缺陷疾病成年患者可能发生的肺部并发症的影像学表现谱对于最大程度地减少诊断延误和改善患者预后非常重要。要点： • 不明原因的支气管扩张，无论是否与闭塞性细支气管炎的CT表现相关，都应引起原发性自身抗体缺乏的潜在诊断。 • 年轻成人严重支气管扩张或气肿时出现的各种曲霉菌病的 CT 证据表明了高 IgE 综合征的肺部并发症。 • 在慢性肉芽肿性疾病患者中，侵袭性曲霉菌病相对常见，通常无症状，有时与非感染性炎症性肺部病变相似或与之相关。© 2023。作者。

Our objective in this review is to familiarize radiologists with the spectrum of initial and progressive CT manifestations of pulmonary complications observed in adult patients with primary immunodeficiency diseases, including primary antibody deficiency (PAD), hyper-IgE syndrome (HIES), and chronic granulomatous disease (CGD). In patients with PAD, recurrent pulmonary infections may lead to airway remodeling with bronchial wall-thickening, bronchiectasis, mucus-plugging, mosaic perfusion, and expiratory air-trapping. Interstitial lung disease associates pulmonary lymphoid hyperplasia, granulomatous inflammation, and organizing pneumonia and is called granulomatous-lymphocytic interstitial lung disease (GLILD). The CT features of GLILD are solid and semi-solid pulmonary nodules and areas of air space consolidation, reticular opacities, and lymphadenopathy. These features may overlap those of mucosa-associated lymphoid tissue (MALT) lymphoma, justifying biopsies. In patients with HIES, particularly the autosomal dominant type (Job syndrome), recurrent pyogenic infections lead to permanent lung damage. Secondary infections with aspergillus species develop in pre-existing pneumatocele and bronchiectasis areas, leading to chronic airway infection. The complete spectrum of CT pulmonary aspergillosis may be seen including aspergillomas, chronic cavitary pulmonary aspergillosis, allergic bronchopulmonary aspergillosis (ABPA)-like pattern, mixed pattern, and invasive. Patients with CGD present with recurrent bacterial and fungal infections leading to parenchymal scarring, traction bronchiectasis, cicatricial emphysema, airway remodeling, and mosaicism. Invasive aspergillosis, the major cause of mortality, manifests as single or multiple nodules, areas of airspace consolidation that may be complicated by abscess, empyema, or contiguous extension to the pleura or chest wall. CLINICAL RELEVANCE STATEMENT: Awareness of the imaging findings spectrum of pulmonary complications that can occur in adult patients with primary immunodeficiency diseases is important to minimize diagnostic delay and improve patient outcomes. KEY POINTS: • Unexplained bronchiectasis, associated or not with CT findings of obliterative bronchiolitis, should evoke a potential diagnosis of primary autoantibody deficiency. • The CT evidence of various patterns of aspergillosis developed in severe bronchiectasis or pneumatocele in a young adult characterizes the pulmonary complications of hyper-IgE syndrome. • In patients with chronic granulomatous disease, invasive aspergillosis is relatively frequent, often asymptomatic, and sometimes mimicking or associated with non-infectious inflammatory pulmonary lesions.© 2023. The Author(s).