Pulmonary Alveolar Proteinosis in Hereditary and Autoimmune Forms With 2 Cases
PEDIATRIC EMERGENCY CARE, vol.36, no.8, 2020 (SCI-Expanded, Scopus)
- Publication Type: Article / Article
- Volume: 36 Issue: 8
- Publication Date: 2020
- Doi Number: 10.1097/pec.0000000000001536
- Journal Name: PEDIATRIC EMERGENCY CARE
- Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, CINAHL, EMBASE, MEDLINE
- Keywords: autoimmune, hereditary, pulmonary alveolar proteinosis, whole-lung lavage
- Dokuz Eylül University Affiliated: Yes
Abstract
Pulmonary alveolar proteinosis (PAP) is a respiratory pathology characterized by the accumulation and increase of surfactant-derived material in the lungs. In clinical practice, PAP may present as the primary form, which includes autoimmune and hereditary PAP, or as the secondary form. Diffuse alveolar radiopacities on chest x-ray and the crazy-paving pattern on high-resolution computed tomography are important, although not specific findings for PAP. Bronchoalveolar lavage biopsy is a diagnostic method, and whole-lung lavage remains the criterion standard for the treatment of PAP. Evidence is required regarding treatment with exogenous anti-granulocyte/macrophage colony-stimulating factor.