Alveolar proteinosis: a cause of immunodeficiency
Ben Hammoud, L.; Dessimoz, C.éd.; Rudaz, D.; Ravussin, P.; Oggier, S.ép.; Tschopp, J.-M.
Revue Medicale Suisse 9(406): 2078-2081
2013
ISSN/ISBN: 1660-9379 PMID: 24383280 Document Number: 671033
Alveolar proteinosis is a rare disease, characterized by accumulation of surfactant in alveoli. Various forms have been identified (congenital, secondary or auto-immune). Treatment is to be reserved for patients that experience moderate to severe symptoms. It requires whole lung lavages, in order to clear the alveoli from the proteinaceous material. Macrophages dysfunction plays a crucial role in the development of the disease and causes immunodeficiency, which in turn can promote opportunistic infections, in patients a priori thought to be immunocompetent.