Pulmonary Alveolar Proteinosis Treatment & Management
- Author: Roger B Olade, MD, MPH; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more...
Management of pulmonary alveolar proteinosis (PAP) depends on the progression of the illness, coexisting infections, and degree of physiological impairment. The standard of care for PAP is mechanical removal of the lipoproteinaceous material by whole-lung lavage. Historically, patients have been treated with systemic steroids, mucolytics (aerosol), and proteinase (aerosol) without much success. Indications for this procedure are a histologic diagnosis in combination with any of the following: (1) an alveolar-arterial oxygen gradient greater than or equal to 40 mm Hg, (2) dyspnea and hypoxemia at rest or with exercise, or (3) a PaO2 of less than 65 mm Hg.
In secondary PAP, appropriate treatment of the underlying cause is warranted. Inhaled and systemic GM-CSF has been shown to be safe and effective in providing a sustained therapeutic effect in autoimmune PAP.
Whole-lung lavage is performed with a double-lumen endotracheal tube designed to allow simultaneous ventilation and lavage. Lung lavage is performed under general anesthesia, and the lung is ventilated briefly with 100% oxygen before lavage with isotonic sodium chloride solution. The standard is lavage with up to 50 L of fluid. Upon completion of the procedure, the lung is suctioned of most of the isotonic sodium chloride solution and allowed to recover before lavaging the other lung. Lung lavage has been performed in hyperbaric chambers, which has made lavage of both lungs possible on the same day. Lung lavage may require several hours.
Rarely, hyperbaric chamber or extracorporeal membrane oxygenation (ECMO) has been used to perform whole-lung lavages in cases of severe hypoxemia.
Lung transplantation is the treatment of choice in patients with congenital PAP and in adult patients with end-stage interstitial fibrosis and cor pulmonale.
Pulmonologist consultation is warranted.
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