WHOLE LUNG LAVAGE IN A CASE OF PULMONARY ALVEOLAR PROTEINOSIS

Abstract

TOPIC: Procedures TYPE: Global Case Reports INTRODUCTION: Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by progressive accumulation of surfactant phospholipids and proteins within alveoli and terminal airways due to decreased surfactant clearance by alveolar macrophage. There are three types,1) congenital2)primary/idiopathic 3) secondary Open lung biopsy with PAS stain is gold standard for diagnosis. Other supportive investigation are chest x ray, HRCT Thorax, GM CSF antibody, hypoxia in ABGA, restrictive or normal PFT.Whole Lung Lavage is mainstay of treatment. GM CSF, plasmapheresis, lung transplantation, supportive care, anti B lymphocyte immunotherapy are other modes of treatment. Some cases shows spontaneous remission. Majority of patients do not require second cycle of WLL. CASE PRESENTATION: 30 year, male, married, non addicted, no co morbidity patient presented to Department of Pulmonary Medicine, B J Medical College, Civil Hospital, Ahmedabad, with chief complaint of progressive difficulty in breathing over 6 to 8 months and dry cough with occasionally whitish expectoration.He works at ship breaking yard.He came with HRCT thorax which had "crazy paving” pattern. On examination he had bilateral fine crepitation and Spo2 86% at room air which reduce to 82% on exertion. So patient hospitalized for further investigations, diagnosis and treatment. DISCUSSION: After admission all routine blood investigations carried out which were within normal limit. On ABGA at room air PaO2 was 66 mmHg. 2D Echo was normal. On PFT restrictive pattern seen. RT PCR for COVID 19 was negative. After exclusion of other causes for HRCT pattern and clinical symptoms bronchoscopy was performed. on bronchoscopy white, frothy proteinosis material seen. On bronchial wash amorphous PAS stained material seen. On culture and sensitivity no organism were cultivated. So after confirmation of diagnosis of pulmonary alveolar proteinosis we planned for whole lung lavage. From HRCT thorax left lung was more involved so on first sitting left sided whole lung lavage performed. With help of left sided double lumen endotracheal tube and single lung ventilation we carried out lavage in 11 sequential cycle. Each cycle took approximately 15-20 minutes. Post procedure patient mechanically ventilated for 16 hours and then extubated without any complication. Post procedure patient improved clinically and radiologically. His right side whole lung lavage is planned after a month. Prior to this whole lung lavage, one patient's WLL was done at our institute. CONCLUSIONS: PAP is relatively rare disease with estimated to be 3.7 case per million individuals. For diagnosis open lung biopsy is the gold standard. Whole lung lavage is mainstay of treatment. This procedure require well pre procedure planning and co ordination among pulmonologist, anesthetist and physiotherapist. REFERENCE #1: Fishman's pulmonary diseases and disorders REFERENCE #2: DeMello De, Lin Z: pulmonary alveolar proteinosis: a review. Pediatr Pathol Mol Med 20:413-432, 2001 REFERENCE #3: Whole lung lavage - technical details, challenges and management of complications. Ahmed Awab, Journal of Thoracic Disease 2017;9(6):1697-1706 DISCLOSURES: no disclosure on file for Ghanshyam Borisagar; no disclosure on file for AMIT DEDUN; No relevant relationships by rajdeep dhandhukiya, source=Web Response No relevant relationships by RAJESH SOLANKI, source=Web Response