Coirier Valentin, Delamaire Flora, Chauvin Pierre, Kerjouan Mallorie, Lederlin Mathieu, Maamar Adel, Jouneau Stéphane
Department of Infectious Diseases and Intensive Care, Rennes University Hospital, Rennes, France.
Department of Respiratory Diseases, Rennes University Hospital, Rennes, France.
Respir Med Case Rep. 2023;42:101825. doi: 10.1016/j.rmcr.2023.101825. Epub 2023 Feb 27.
Autoimmune pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the alveoli accumulation of surfactants proteins and lipids, which diagnosis is confirmed by the presence of GM-CSF antibodies in serum. PAP can be evoked when its characteristic images on chest computed-tomography (CT) are present: bilateral and multifocal ground-glass opacities and crazy-paving appearance. Patients with PAP are at an increased risk of opportunistic infections caused by Nocardia, mycobacteria and fungal pathogens due to impaired processing of pulmonary surfactant. We here report a typical case of newly diagnosed autoimmune PAP, with initial indication to realize a whole-lung lavage. Despite this treatment the patient presented a marked clinical worsening, with increasing need for oxygen and finally the need for mechanical ventilation. The chest CT was controlled and found to be typical of PAP, while the search for opportunistic infections remained negative. Finally, SARS-CoV-2 PCR was performed on bronchoalveolar lavage fluid, and was positive, whereas it had previously been negative twice. Our case report highlights the difficulty of distinguishing SARS-CoV-2 infection in the context of PAP, as the chest CT features are similar. We believe that a SARS-CoV-2 RT-PCR should be systematically realized in case of respiratory deterioration in PAP patients.
自身免疫性肺泡蛋白沉积症（PAP）是一种罕见疾病，其特征为肺泡内表面活性物质蛋白和脂质蓄积，血清中GM-CSF抗体的存在可确诊该病。当胸部计算机断层扫描（CT）出现其特征性影像时可诱发PAP：双侧多灶性磨玻璃影及铺路石样表现。由于肺表面活性物质处理受损，PAP患者发生诺卡菌、分枝杆菌和真菌病原体所致机会性感染的风险增加。我们在此报告一例新诊断的自身免疫性PAP典型病例，最初的治疗方案是进行全肺灌洗。尽管进行了该治疗，患者的临床症状仍显著恶化，对氧气的需求增加，最终需要机械通气。复查胸部CT符合PAP表现，而机会性感染检查结果为阴性。最后，对支气管肺泡灌洗液进行了SARS-CoV-2聚合酶链反应（PCR）检测，结果呈阳性，而此前两次检测均为阴性。我们的病例报告强调了在PAP背景下区分SARS-CoV-2感染的困难，因为胸部CT特征相似。我们认为，PAP患者出现呼吸恶化时应常规进行SARS-CoV-2逆转录PCR检测。
