OrphaChat — a Rare Disease Podcast

Autoimmune Pulmonary Alveolar Proteinosis


Listen Later

These documents examine autoimmune pulmonary alveolar proteinosis (aPAP), a rare respiratory disorder where a surfactant buildup in the lungs obstructs oxygen absorption. This condition is primarily driven by autoantibodies that neutralise the proteins responsible for clearing lung debris, leading to symptoms like shortness of breath and chronic fatigue. Whole-lung lavage, a procedure involving the physical washing of the lungs under general anaesthesia, remains the conventional treatment for removing these accumulations. Recent clinical research focuses on molgramostim, an inhaled therapy that aims to restore normal lung function by addressing the underlying immune dysfunction. Study results indicate that this inhaled treatment can significantly improve gas exchange and the physical capacity of patients over long-term periods. Additionally, the texts provide medical guidelines, diagnostic criteria, and case studies detailing how the disease interacts with other health issues such as infections and blood disorders.

...more
View all episodesView all episodes
Download on the App Store

OrphaChat — a Rare Disease PodcastBy Robin Hendel, MD