Pulmonary alveolar proteinosis: a new autoimmune disease. 2005

Ulrich Costabel, and Josune Guzman
Medical Faculty, University of Duisburg-Essen, and Department of Pneumology/Allergy, Ruhrlandklinik, Essen, Germany. erj.costabel@t-online.de

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.

UI MeSH Term Description Entries
D007150 Immunohistochemistry Histochemical localization of immunoreactive substances using labeled antibodies as reagents. Immunocytochemistry,Immunogold Techniques,Immunogold-Silver Techniques,Immunohistocytochemistry,Immunolabeling Techniques,Immunogold Technics,Immunogold-Silver Technics,Immunolabeling Technics,Immunogold Silver Technics,Immunogold Silver Techniques,Immunogold Technic,Immunogold Technique,Immunogold-Silver Technic,Immunogold-Silver Technique,Immunolabeling Technic,Immunolabeling Technique,Technic, Immunogold,Technic, Immunogold-Silver,Technic, Immunolabeling,Technics, Immunogold,Technics, Immunogold-Silver,Technics, Immunolabeling,Technique, Immunogold,Technique, Immunogold-Silver,Technique, Immunolabeling,Techniques, Immunogold,Techniques, Immunogold-Silver,Techniques, Immunolabeling
D008297 Male Males
D011649 Pulmonary Alveolar Proteinosis A PULMONARY ALVEOLI-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and DYSPNEA. This disease is often related to, congenital or acquired, impaired processing of PULMONARY SURFACTANTS by alveolar macrophages, a process dependent on GRANULOCYTE-MACROPHAGE COLONY-STIMULATING FACTOR. Alveolar Proteinoses, Pulmonary,Alveolar Proteinosis, Pulmonary,Proteinoses, Pulmonary Alveolar,Proteinosis, Pulmonary Alveolar,Pulmonary Alveolar Proteinoses
D012075 Remission, Spontaneous A spontaneous diminution or abatement of a disease over time, without formal treatment. Spontaneous Healing,Spontaneous Regression,Spontaneous Remission,Healing, Spontaneous,Regression, Spontaneous,Spontaneous Healings,Spontaneous Regressions
D012129 Respiratory Function Tests Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc. Lung Function Tests,Pulmonary Function Tests,Function Test, Pulmonary,Function Tests, Pulmonary,Pulmonary Function Test,Test, Pulmonary Function,Tests, Pulmonary Function,Function Test, Lung,Function Test, Respiratory,Function Tests, Lung,Function Tests, Respiratory,Lung Function Test,Respiratory Function Test,Test, Lung Function,Test, Respiratory Function,Tests, Lung Function,Tests, Respiratory Function
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001327 Autoimmune Diseases Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides. Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune
D001707 Biopsy, Needle Removal and examination of tissue obtained through a transdermal needle inserted into the specific region, organ, or tissue being analyzed. Aspiration Biopsy,Puncture Biopsy,Aspiration Biopsies,Biopsies, Aspiration,Biopsies, Needle,Biopsies, Puncture,Biopsy, Aspiration,Biopsy, Puncture,Needle Biopsies,Needle Biopsy,Puncture Biopsies

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