Pulmonary Alveolar Proteinosis An under diagnosed entity
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.
References
2. Griese M, Brasch F, Aldana V R, Cabrera M M, Goelnitz U, Ikonen E, et al. Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis. Clin Genet. 2010;77(2):119-30.
3. Xue Y, Han Y, Li T, Chen S, Zhang J, Pan J, et al. Pulmonary alveolar proteinosis as a terminal complication in a case of myelodysplastic syndrome with idic(20q-). Acta Haematol. 2010;123(1):55-8.
4. Wardwell N R Jr, Miller R, Ware L B. Pulmonary alveolar proteinosis associated with a disease-modifying antirheumatoid arthritis drug. Respirology. 2006;11(5):663-5.
5. Fang SC, Lu KH, Wang CY, Zhang HT, Zhang YM. Elevated tumor markers in patients with pulmonary alveolar proteinosis. Clin Chem Lab Med. 2013;1-6.
6. Ramirez J: Pulmonary alveolar proteinosis. Treatment by massive bronchopulmonary lavage. Arch Intern Med 1967; 119:147-156.
7. Michaud G, Reddy C, Ernst A: Whole-lung lavage for pulmonary alveolar proteinosis. Chest 2009, 136:1678- 1681.
8. MG De Vega, A Sanchez – Palenchia, A. Ramirez, S Cevera and J Aneiroz. GM-CSF Therapy in Pulmonary Alveolar Proteinosis. Thorax 2002; 57(9): 837
