Pulmonary alveolar proteinosis: a new autoimmune disease
Ulrich Costabel1, Josune Guzman
1Medical Faculty, University of Duisburg-Essen, and Department of Pneumology/Allergy, Ruhrlandklinik, Essen, Germany. erj.costabel@t-online.de
Abstract:
Pulmonary alveolar proteinosis is a rare disorder characterised by abundant accumulation of surfactant-derived phospholipids and protein components in the alveoli and distal airways. In this review we focus the discussion on acquired (idiopathic or primary) alveolar proteinosis. We report the studies that developed the concept that this disorder is an autoimmune disease caused by auto-antibodies against GM-CSF. The clinical management of the patients is also described.
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