Pulmonary Alveolar Proteinosis An under diagnosed entity
Journal Title: The Indian Practitioner - Year 2014, Vol 67, Issue 9
Abstract
Pulmonary alveolar proteinosis (PAP) is a rare, congenital or acquired disease characterised by impaired surfactant clearance that leads to accumulation of an amorphous, lipoproteinaceous material in the alveoli, largely made up of surfactant protein (SP) - A,B,C,D which are Periodic Acid Schiff (PAS) positive. A 53 year old male presented with anorexia, weight loss, progressive dyspnoea on exertion & cough with whitish mucoid expectoration. Investigations showed severe hypoxia and elevated serum LDH. X ray chest had Bat-wing appearance with bilateral infiltration with spared lung apices. HRCT chest showed bilateral alveolar filling with ‘crazy-pavement’ appearance with well preserved interlobular and alveolar architecture suggestive of PAP. Bronchoscopic aspiration of bronchoalveolar lavage (BAL) fluid examination confirmed the diagnosis of PAP. Patient improved with alveolar aspiration.
Authors and Affiliations
N. N Redkar, K. J. Rawat, V Agarwal, P Kolhe, J Shah
Keywords
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