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Pulmonary alveolar proteinosis
Tisha Wang1, Catherine A Lazar, Michael C Fishbein
1Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Internal Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Seminars in Respiratory and Critical Care Medicine
|September 25, 2012
Summary
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder caused by surfactant buildup. Autoimmune PAP, the most common form, stems from antibodies affecting surfactant clearance.
Area of Science:
- Pulmonology
- Rare Diseases
- Immunology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation in alveoli, impairing gas exchange.
- PAP's clinical course varies, from spontaneous resolution to fatal respiratory failure.
- Excessive surfactant accumulation drives PAP, with autoimmune factors being the most common cause in humans.
Purpose of the Study:
- To review the pathophysiology, clinical presentation, diagnosis, and treatment of Pulmonary Alveolar Proteinosis (PAP).
- To highlight the role of granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies in autoimmune PAP.
- To discuss diagnostic criteria and current therapeutic strategies, including whole lung lavage and emerging GM-CSF therapies.
Main Methods:
- Literature review of PAP pathophysiology, clinical features, diagnostic modalities, and treatment options.
- Analysis of the role of autoantibodies to GM-CSF in autoimmune PAP.
- Evaluation of diagnostic approaches including imaging (CT scan) and bronchoalveolar lavage findings.
Main Results:
- Autoimmune PAP, caused by anti-GM-CSF autoantibodies, is the predominant form, leading to impaired surfactant clearance.
- Inherited and secondary forms of PAP are rarer, linked to genetic mutations or reduced macrophage function, respectively.
- Clinical symptoms include dyspnea and cough, with characteristic imaging findings like 'crazy paving' on HRCT.
Conclusions:
- PAP diagnosis relies on clinical, imaging, and laboratory findings, often avoiding the need for lung biopsy.
- Whole lung lavage remains a primary treatment, though not always necessary.
- GM-CSF therapy shows promise for autoimmune PAP, but optimal treatment parameters require further research.
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