Introduction:
Pulmonary alveolar proteinosis is a rare disease that is characterized by accumulation of surfactant and phospholipids in the pulmonary alveoli. The current mainstay of treatment is whole lung lavage. There have been rare reports that have revealed the effectiveness of N-acetylcysteine on a secondary PAP.
Case presentation:
A 45-year old man complained of shortness of breath and a productive cough with white sputum. He inhaled stone powder as an occupational hazard. CT scan of his chest radiograph showed ground glass changes superimposed on a reticular pattern as the so-called 'crazy paving' pattern. Lung biopsy revealed alveolar proteinaceous material with positive PAS stain. He eventually was diagnosed as PAP. He refused a whole lung lavage therapy, so the patient was rendered N-acetylcysteine as an antioxidant, enhanced immunotherapy and anti-infective treatment. His clinical symptoms and radiological manifestation improved gradually. No substantial adverse reactions were reported.
Conclusions:
Persistent oral N-acetylcysteine may be an alternative treatment option for secondary PAP.