Abstract
Pulmonary alveolar proteinosis (PAP) is a rare disease with mostly due to autoimmune toward granulocyte-macrophage colony-stimulating factor. In some conditions, PAP followed with secondary infection. A 34-year-old woman came with progressive shortness of breath, chronic dry cough, and mild fever. The chest High-Resolution Computed Tomography showed ground-glass opacity with septal reticulation or known as the crazy-paving pattern, and a cavity on the upper lobe of the left lung. The patient underwent bronchoscopy for diagnostic and therapeutic measures and found milky appearance bronchoalveolar lavage fluid (BALF). The serum galactomannan came out positive. Fungal infection detected from the BALF culture, Aspergillus fumigatus, hence fulfilling the diagnosis of PAP with probable invasive pulmonary aspergillosis. The patient showed clinical improvement after undergoing whole lung lavage and given anti-fungal medications.
Highlights
Pulmonary alveolar proteinosis (PAP) is a rare disease marked by deposits of surfactant components in the alveolus (1)
More than 90% of PAP cases are autoimmune PAP with an increase of antibody towards granulocyte-macrophage colony-stimulating factor (GM-CSF), a cytokine affecting the final differentiation of alveolar macrophage and decreasing the function of circulating neutrophils, so besides causing surfactant deposits, it can increase the risk of infection (1,2)
It is hard to find the etiology of PAP due to limitations in supporting examinations needed such as anti-GMCSF antibody, GM-CSF level, or genetic testing
Summary
Pulmonary alveolar proteinosis (PAP) is a rare disease marked by deposits of surfactant components in the alveolus (1). Based on the current immunocompromised status of the patient (taking high dose of corticosteroid for a long time), a cavity in the chest HRCT, positive serum galactomannan, and positive Aspergillus culture from BALF, she was subsequently diagnosed with PAP with probable invasive pulmonary aspergillosis and treated with anti-fungal itraconazole of 150 mg once daily, for 3 months. Her symptoms and signs showed further improvements each month. Limited facilities for diagnosis and management of PAP are the obstacles for this patient
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