Pulmonary alveolar proteinosis in Turkey: a review of twenty four cases

Background: This study aims to investigate the overall characteristics of the patients with pulmonary alveolar proteinosis (PAP) in Turkey. Methods: Between January 1958 and July 2010, national and international databases were scanned to reach articles on PAP in Turkey and full texts were reviewed. Demographic characteristics of patients, clinical data, laboratory findings, imaging studies, diagnostic methods and treatments were recorded. Results: Twenty-two articles reporting 24 cases were found in the literature. The mean age was 21 +/- 14 years (range, 1 day to 47 years). Thoracic computed tomography revealed crazy paving appearance in nine patients, ground glass appearance in five patients, and infection in two patients. Nine patients underwent transbronchial biopsy (TBB), while 10 underwent open lung biopsy (OLB). Diagnosis was based on autopsy in three cases, bronchoalveolar lavage fluid (BALE) in two cases, TBB in four cases, OLB in nine cases, BALF and TBB in three cases, BALF and electron microscopy (EM) in one case, BALF, EM and,TBB in one case, and BALF, EM and OLB in one case. Whole lung lavage was performed on six cases, while four cases underwent segmental bronchial lavage. One case was treated with intravenous immunoglobulin, while one case was treated with granulocyte colony stimulating factor. Five cases died during hospitalization. Chest X-ray revealed improved hypoxia in four cases, improved lung function test results in three cases and regressed lesions in five cases. Conclusion: Our study results suggest that diagnostic and management approaches for Turkish patients with PAP are consistent with the published materials in the literature.