Atypical pulmonary alveolar proteinosis - A diagnostic challenge

Case Report

Author Details : Pratap Upadhya, Ravindra Chary, Ramya Priya A*, Sree Rekha

Volume : 6, Issue : 2, Year : 2021

Article Page : 130-132

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Pulmonary alveolar proteinosis (PAP) is a rare syndrome, characterized by ground-glass opacities associated with reticulations giving a characteristic crazy paving appearance which is diagnostic but not pathognomonic in imaging. A 47-year-old male presented with breathlessness and dry cough. Arterial blood gas (ABG) showed hypoxemia and respiratory alkalosis with Alveolar- arterial (A-a) O2 gradient of 82. HRCT thorax suggestive of crazy paving pattern along with solitary nodules of low density in right upper and lower lobes giving suspicion of malignancy with secondary PAP. The clinical probability of malignancy was moderate hence surgical lung biopsy was performed which suggested PAP. Granulocytemacrophage colony-stimulating factor (GM-CSF) autoantibody concentration was 118.7mcg/ml suggestive of autoimmune PAP. Patient was treated with inhalational GM-CSF with significant clinical response ((A-a) O2 gradient improved to 24). Though crazy paving is characteristic for PAP, speculated low density atypical multi nodular appearance may also be possible which mandates biopsy for confirmation.

Keywords: Fiber optic Bronchoscopy (FOB), Hypoxemia, Pulmonary alveolar proteinosis (PAP).

How to cite : Upadhya P , Chary R , Ramya Priya A, Rekha S , Atypical pulmonary alveolar proteinosis - A diagnostic challenge. IP Indian J Immunol Respir Med 2021;6(2):130-132

Copyright © 2021 by author(s) and IP Indian J Immunol Respir Med. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License (

Article History

Received : 27-04-2021

Accepted : 15-05-2021

Available online : 07-06-2021

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