Pulmonary alveolar proteinosis: a new autoimmune disease.
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.
Costabel
Ulrich
U
Medical Faculty, University of Duisburg-Essen, and Department of Pneumology/Allergy, Ruhrlandklinik, Essen, Germany. erj.costabel@t-online.de
Guzman
Josune
J
D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D001707:Biopsy, Needle; D017322:Clinical Trials, Phase II as Topic; D005260:Female; D005500:Follow-Up Studies; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D011649:Pulmonary Alveolar Proteinosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D012075:Remission, Spontaneous; D012129:Respiratory Function Tests; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D016178:Granulocyte-Macrophage Colony-Stimulating Factor