Diagnosis and treatment of pulmonary alveolar proteinosis in adults
- Edward D Chan, MD
Edward D Chan, MD
- Professor of Medicine
- National Jewish Health
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
Pulmonary alveolar proteinosis (PAP), also known as pulmonary alveolar phospholipoproteinosis, is a diffuse lung disease characterized by the accumulation of amorphous, periodic acid-Schiff (PAS)-positive lipoproteinaceous material in the distal air spaces [1-3].
The most common symptoms are dyspnea and cough. Radiographic imaging typically reveals bilateral symmetric alveolar opacities located centrally in mid and lower lung zones, often in a "bat wing" distribution. Three forms of PAP are recognized: congenital, secondary, and acquired (table 1). A more detailed discussion of the etiology and clinical manifestations of PAP is provided separately. (See "Clinical manifestations and etiology of pulmonary alveolar proteinosis in adults".)
This review will provide an approach to the diagnosis and treatment of PAP. General approaches to adult and pediatric interstitial lung disease are described separately. (See "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing" and "Approach to the infant and child with diffuse lung disease (interstitial lung disease)".)
EVALUATION AND DIAGNOSIS
Our diagnostic algorithm for suspected PAP is shown in the figure (algorithm 1). When findings suggestive of PAP are noted on HRCT scan in patients with a compatible clinical presentation, we proceed to fiberoptic bronchoscopy to obtain bronchoalveolar lavage (BAL) fluid and, when possible, transbronchoscopic biopsies. Unless contraindicated, transbronchoscopic biopsies should be obtained following the BAL procedure. The diagnosis of PAP is confirmed with a positive PAS stain from BAL or transbronchial biopsy. To exclude the presence of concurrent infection, special stains and cultures for opportunistic infections (eg, Nocardia, mycobacteria, fungi) are also obtained during bronchoscopy. (See "Clinical manifestations and etiology of pulmonary alveolar proteinosis in adults", section on 'Associated infections'.)
A transbronchial or surgical lung biopsy is the definitive diagnostic test for PAP. In many cases, the diagnostic accuracy of PAS staining of fluid obtained by BAL and tissue obtained by transbronchial biopsy has obviated the need for open or thoracoscopic lung biopsy when clinical and CT findings are consistent with the condition [1-7]. As an example, in the largest series, the diagnosis was made by HRCT and BAL in 59 percent; HRCT, BAL and transbronchial biopsy in 34 percent; and video-assisted transthoracic biopsy in 7 percent .
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