Pulmonary Alveolar Proteinosis
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Slowly progressive dyspnea, now oxygen dependent. Bilateral lung transplant 6 years ago for bronchiectasis associated with ciliary dyskinesia Numerous medications, including everolimus.
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Geographic crazy paving, gradually increasing in extent over serial CT scans.
1 case question available
Bronchoscopy: large volume of proteinaceous material aspirated.
Bronchoscopic biopsies: Alveolar spaces within the parenchymal fragments are filled with slightly granular or slightly frothy appearing proteinaceous material within which there are scattered, degenerate appearing, alveolar macrophages. No evidence of acute cellular rejection, ISHLT grade A0/B0.
Rhinovirus PCR detected, otherwise no infection.
Everolimus ceased as this drug has been associated with PAP.
PAP can be treated by bronchial lavage, taking several hours. Unfortunately the patient is not well enough.