Treatment and prognosis 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: primary (autoimmune, receptor variants), secondary (hematologic/immunologic disease, inhalational exposures, metabolic), and congenital (surfactant production disorders) (table 1).
The treatment and prognosis of PAP will be reviewed here. General approaches to adult and pediatric interstitial lung disease and the clinical manifestations and diagnosis of pulmonary alveolar proteinosis in adults 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)" and "Causes, clinical manifestations, and diagnosis of pulmonary alveolar proteinosis in adults".)
ASSESSMENT OF DISEASE SEVERITY
In order to determine the appropriate treatment, severity of PAP is assessed using a combination of symptoms, serum level of lactic dehydrogenase (LDH), titer of serum antibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) in autoimmune PAP, pulmonary function tests, and extent of opacities on high resolution computed tomography (HRCT) imaging . Pulmonary function tests, including lung volumes, diffusing capacity, and six-minute walk, are used to assess the degree of impairment and to provide a baseline for long-term monitoring. (See "Causes, clinical manifestations, and diagnosis of pulmonary alveolar proteinosis in adults", section on 'Pulmonary function tests'.)
Serial measurements of bronchoalveolar lavage (BAL) or serum antibodies to GM-CSF may prove useful in monitoring disease activity and response to treatment in autoimmune PAP, although further study of this approach is needed [5,6]. In one study, BAL fluid levels of anti-GM-CSF antibodies correlated better with the severity of PAP compared to serum titers .
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- ASSESSMENT OF DISEASE SEVERITY
- TREATMENT OF AUTOIMMUNE PAP
- Supportive care
- Asymptomatic or mild disease
- Moderate-to-severe respiratory impairment
- - Whole lung lavage
- - Recombinant GM-CSF
- Refractory disease
- - Rituximab
- - Therapeutic plasma exchange
- - Clinical trials
- - Lung transplantation
- Future directions
- Ineffective therapies
- TREATMENT OF OTHER TYPES OF PAP
- INFECTIOUS COMPLICATIONS
- SUMMARY AND RECOMMENDATIONS