SESSION TITLE: Medical Student/Resident Chest Infections Posters
SESSION TYPE: Med Student/Res Case Rep Postr
PRESENTED ON: October 18-21, 2020
INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of amorphous lipoproteins in the distal air spaces which impairs gas exchange, causing hypoxia. Incidence is around 0.2 cases per million and it is more commonly seen in males (1). Herein, we describe a rare case of PAP in a middle-aged female who was subsequently affected with COVID-19
CASE PRESENTATION: A 47-year-old Hispanic lady with a history of hypertension and obstructive sleep apnea presented to the outpatient clinic with worsening dyspnea on exertion for 7 months and chronic cough with whitish sputum production. She was a homemaker and had no family history of any lung disease. Physical examination showed that the patient was dyspneic. The lung examination was remarkable for inspiratory crackles in bilateral lung fields. Pulmonary function tests revealed reduced FVC: 1.79L (64% of predicted) and FEV1: 1.54L (68% of predicted) with a normal FEV1/FVC ratio of 82%. DLCO was 11.2 mL/min/mmHg (55%) suggestive of moderate restrictive disease and a moderate reduction in diffusion capacity. A chest radiograph revealed diffuse interstitial opacities, computed tomography (CT) thorax confirmed the same. Fiberoptic bronchoscopy biopsies were positive for periodic acid of Schiff (PAS) lipo-proteinaceous material. Granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies were positive. Rituximab was started and she underwent therapeutic whole lung lavage (WLL) which relieved her symptoms. Eight months later, she presented to the emergency department with similar complaints. Initially, it was thought to be PAP flare, but vitals were remarkable for tachycardia, a saturation of 72% on room air, and a fever of 101 F. Lung examination revealed decreased breath sounds bilaterally. Laboratory investigations showed leukocytosis, elevated d-dimer, and ferritin. The patient was intubated and transferred to the intensive care unit. CT pulmonary angiogram revealed bilateral ground-glass opacities and COVID-19 was positive. She was on vasopressors and treated with hydroxychloroquine, azithromycin, remdesivir, and tocilizumab. Later, she developed acute respiratory distress syndrome and died of cardiac arrest.
DISCUSSION: PAP can be complicated with infection with Nocardia species, Mycobacterium species, C. neoformans, H. capsulatum, P. carinii, and viruses due to impaired macrophage function and impaired host defense which favors the growth of microorganisms. No case of COVID19 pneumonia has been reported in a patient with PAP to the best of our knowledge(2).
CONCLUSIONS: The disease course of PAP varies, and the prognosis is unpredictable. WLL is the standard therapy and the 5-year survival of autoimmune PAP is 95% (3). Our patient was recently diagnosed with PAP. She had responded well to previous WLL and the prognosis was good until she was infected by COVID19, implying that it can have deleterious outcomes in PAP.
Reference #1: Carrington JM, Hershberger DM. Pulmonary Alveolar Proteinosis. [Updated 2019 Apr 3]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482308/
Reference #2: Serpil Tekgül, Semra Bilaceroglu, Sevket Ozkaya,∗ Ayse Coskun, Berna Komurcuoglu, and Ali Kadri Cirak. Pulmonary alveolar proteinosis and superinfection with pulmonary tuberculosis in a case. Respir Med Case Rep. 2012; 5: 25–28. doi: 10.1016/j.rmedc.2011.11.003. PMID: 26057858
Reference #3: Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, Epaud R, Crestani B. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011 Jun;20(120):98-107. PMID: 21632797 DOI: 10.1183/09059180.00001311
DISCLOSURES: No relevant relationships by Ankit Agrawal, source=Web Response
No relevant relationships by Sanya Chandna, source=Web Response
No relevant relationships by Monarch Shah, source=Web Response