Open AccessAtypical pulmonary alveolar proteinosis - A diagnostic challenge
Author(s) -
Pratap Upadhya,
Ravindra Chary,
Ramya Priya A
Publication year - 2021
Publication title -
indian journal of immunology and respiratory medicine
Language(s) - English
Resource type - Journals
ISSN - 2456-012X
DOI - 10.18231/j.ijirm.2021.028
Subject(s) - pulmonary alveolar proteinosis , medicine , malignancy , pathognomonic , hypoxemia , pathology , biopsy , lung , radiology , disease
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) O 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. Granulocyte-macrophage 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) O 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.