What is it about?
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease that concerns pulmonary interstitium through an abnormal and excessive accumulation of surfactant inside the alveoli, which can affect gas exchange and can lead to dyspnea and limited efforts.1 It is a diffuse pulmonary disease of unknown etiology and characterized by the accumulation in the distal airspace of a positive amorphous PAS lipoproteinate material that does not alter the pulmonary architecture.2
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Why is it important?
Pulmonary alveolar proteinosis is a rare disease that poses difficulties in diagnosis through uncharacteristic symptoms.
Perspectives
In moderate or severe forms, total bronchoalveolar lavage is recommended. Systemic corticotherapy or immunosuppression is not indicated.
MD, BS, MHA, LLM, FAPSR, FCCP Petru Emil Muntean
European Respiratory Society
Read the Original
This page is a summary of: Clinical Aspects and Evolution under Current Treatments on Pulmonary Alveolar Phospholipoproteinosis Patients, Folia Medica, March 2019, De Gruyter,
DOI: 10.2478/folmed-2018-0048.
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