Wu Xiao-Kang, Lin Quan
Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325015, Zhejiang Province, China.
World J Clin Cases. 2021 Apr 26;9(12):2874-2883. doi: 10.12998/wjcc.v9.i12.2874.
Pulmonary alveolar proteinosis (PAP) is a pulmonary syndrome wherein large volumes of phospholipid and protein-rich surfactants accumulate within the alveoli. PAP forms include primary (auto-immune PAP), secondary, and congenital. Nocardiosis is a form of suppurative disease induced upon infection with bacteria of the genus. Clinically, cases of PAP complicated with infections are rare, regardless of form. Unfortunately, as such, they are easily overlooked or misdiagnosed. We describe, here, the case of a patient suffering from simultaneous primary PAP and nocardiosis.
A 45-year-old Chinese man, without history of relevant disease, was admitted to our hospital on August 8, 2018 to address complaints of activity-related respiratory exertion and cough lasting over 6 mo. Lung computed tomography (CT) revealed diffuse bilateral lung infiltration with local consolidation in the middle right lung lobe. Subsequent transbronchial lung biopsy and CT-guided lung biopsy led to a diagnosis of primary PAP (granulocyte-macrophage colony-stimulating factor antibody-positive) complicated with nocardiosis (periodic acid-Schiff-positive). After a 6 mo course of anti-infective treatment (sul-famethoxazole), the lesion was completely absorbed, such that only fibrous foci remained, and the patient exhibited significant symptom improvement. Follow-up also showed improvement in pulmonary function and the CT imaging findings of PAP. No whole-lung lavage has been conducted to date. This case highlights that active anti-nocardia treatment may effectively improve the symptoms and alleviate PAP in patients with PAP and nocardia, possibly reducing the need for whole-lung lavage.
When evaluating patients presenting with PAP and pulmonary infections, the potential for nocardiosis should be considered.
肺泡蛋白沉积症（PAP）是一种肺部综合征，大量富含磷脂和蛋白质的表面活性剂在肺泡内积聚。PAP的形式包括原发性（自身免疫性PAP）、继发性和先天性。诺卡菌病是由诺卡菌属细菌感染引起的一种化脓性疾病。临床上，无论何种形式，PAP合并感染的病例都很罕见。不幸的是，正因如此，它们很容易被忽视或误诊。在此，我们描述一例同时患有原发性PAP和诺卡菌病的患者。
一名45岁的中国男性，无相关疾病史，于2018年8月8日因活动相关的呼吸费力和咳嗽持续超过6个月而入院。肺部计算机断层扫描（CT）显示双侧肺部弥漫性浸润，右肺中叶局部实变。随后的经支气管肺活检和CT引导下肺活检确诊为原发性PAP（粒细胞-巨噬细胞集落刺激因子抗体阳性）合并诺卡菌病（过碘酸-希夫染色阳性）。经过6个月的抗感染治疗（磺胺甲恶唑），病变完全吸收，仅留下纤维病灶，患者症状明显改善。随访还显示PAP的肺功能和CT影像学表现有所改善。迄今为止尚未进行全肺灌洗。该病例表明，积极的抗诺卡菌治疗可能有效改善PAP合并诺卡菌病患者的症状并减轻PAP，可能减少全肺灌洗的需求。
在评估患有PAP和肺部感染的患者时，应考虑诺卡菌病的可能性。
