Abstract

Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized with surfactant accumulation in the alveoli that distrupt the gas exchange. Asymptomatic PAP cases can be followed radiologically and functionally, whereas in cases with respiratory failure, total lung lavage (TAL) is the first treatment option. Here we shared three PAP cases who underwent TAL.

Cases: One patient was male, two were female, mean age was 36years and follow-up period was 13years. Only one case reported intense mold exposure.  They had progressive dyspnea and dry cough at the admission. Mean duration of the complaints was 16 months. Two patients had respiratory failure and one patient had oxygen desaturation during exercise. At admission, only one case could be evaluated functionally (FVC:66%, DLCO:26%). The others could not be evaluated due to mental retardation and severe respiratory failure. All cases had bilateral crazy paving pattern in HRCT and also they had  milky and PAS positive with bronchoalveolar lavage. Anti-GM-CSF autoantibody was high in one case. TAL indications were respiratory failure in two cases, severe symptoms, extensive radiological involvement and desaturation during exercise in the third case. After TAL, two cases did not need oxygen anymore, one case had an increase in DLCO and FVC (FVC: 77%, DLCO: 53%). In the follow-up period, one patient underwent TAL two more times and the inhaled GMC-SF therapy was initiated.

Conclusions: Current evidence suggests that TAL is still standard therapy for PAP. However, inhaled GM-CSF therapy is an alternative for patients who do not benefit from TAL and / or patients with autoantibody positivity.