Boujaoude Ziad, Arya Rohan, Rafferty William, Dammert Pedro
Department of Pulmonary and Critical Care Medicine, Cooper University Hospital, Camden, New Jersey, USA.
BMJ Case Rep. 2013 Jun 7;2013:bcr2013008905. doi: 10.1136/bcr-2013-008905.
Common variable immunodeficiency (CVID) is the most common of the primary immunodeficiency disorders. Pulmonary manifestations are characterised by recurrent rhinosinusitis, respiratory tract infections and bronchiectasis. Less commonly the lung may be affected by lymphoid disorders and sarcoid-like granulomas. Organising pneumonia (OP) is a rare pulmonary manifestation. We report the case of a 32-year-old woman with CVID who presented with fever, dyspnoea and persistent lung infiltrates despite antibiotic therapy. CT of the chest showed bilateral patchy alveolar infiltrates. Pulmonary function tests revealed moderate restriction and reduction in diffusion capacity. Initial bronchoscopy with transbronchial biopsies did not yield a diagnosis but surgical lung biopsies identified OP. Significant clinical, radiographic and physiological improvement was achieved after institution of corticosteroid therapy.
普通可变型免疫缺陷（CVID）是最常见的原发性免疫缺陷疾病。肺部表现的特征为复发性鼻-鼻窦炎、呼吸道感染和支气管扩张。较少见的情况是，肺部可能受淋巴系统疾病和类肉瘤样肉芽肿影响。机化性肺炎（OP）是一种罕见的肺部表现。我们报告一例32岁患有CVID的女性病例，该患者出现发热、呼吸困难，尽管接受了抗生素治疗，但肺部浸润持续存在。胸部CT显示双侧斑片状肺泡浸润。肺功能测试显示有中度受限和弥散功能降低。最初的支气管镜检查及经支气管活检未得出诊断，但外科肺活检确诊为OP。给予皮质类固醇治疗后，临床、影像学和生理学方面均有显著改善。
