PULMONARY ALVELOLAR PROTEINOSIS : A case report

Dr. Harshvardhan Singh Rathore; Dr. Pratik Patil; Dr. Madan Manmohan; Dr. Om Tavri; Dr. Neeti Mathur

Volume : VIII, Issue : VIII, August - 2019

PULMONARY ALVELOLAR PROTEINOSIS : A case report

Dr. Harshvardhan Singh Rathore, Dr. Pratik Patil, Dr. Madan Manmohan, Dr. Om Tavri, Dr. Neeti Mathur

Abstract :

Pulmonary alveolar proteinosis(PAP) is a rare diffuse interstitial lung disease with a prevalence of 1 per 10,00,000. It was first described in 1958 by Rosen et al. It is characterized by abnormal accumulation of pulmonary surfactant in the alveolar space, which in turn impairs gaseous exchange leading to a severe hypoxemia . Pulmonary surfactant is an insoluble proteinaceous material that is rich in lipids and stains positive with periodic acid–Schiff (PAS). It is caused due to deficiency in granulocyte macrophage–colony stimulating factor (GM–CSF), as a result of the anti–GM–CSF antibody production, which ultimately leads to the accumulation of surfactant in the alveolar space. It is usually present in young and middle–aged adults (20–50 years of age) . It is associated with smoking and has a male predilection (M:F of ~3:1) Its clinical course is variable, ranging from complete remission to fatal respiratory failure. On imaging, it is classically associated with� Crazy paving pattern� on HRCT, although it is a rare cause of this non–specific finding. Whole lung lavage was the gold standard therapy in PAP until the advent of GM–CSF. We here report a case of 52 year old Indian male who presented to OPD with a history of long standing cough with whitish sputum cough , progressive shortness of eath on exertion and multiple joint pain since 6 month