Goodpasture’S Syndrome

(Goodpasture’s syndrome, anti-GBM antibody disease)

Goodpasture’s syndrome, a subtype of the pulmorenales syndrome is an autoimmune disease with alveolar hemorrhage and glomerulonephritis which is caused by circulating anti-GBM antibody. Most commonly, a Goodpasture’s syndrome develops in genetically predisposed smokers, but exposure to hydrocarbons and respiratory viral infections are other possible triggers. The symptoms is to dyspnea, coughing, fatigue, hemoptysis, and hematuria. A Goodpasture’s syndrome is suspected in patients with hemoptysis or hematuria and detected by the detection of anti-GBM antibodies in the blood or kidney biopsy samples. The prognosis is good when treatment begins before developing a pulmonary or renal insufficiency. The therapy includes plasma exchange, corticosteroids and immunosuppressive agents such as cyclophosphamide.

Goodpasture’s syndrome, a subtype of the pulmorenales syndrome is an autoimmune disease with alveolar hemorrhage and glomerulonephritis which is caused by circulating anti-GBM antibody. Most commonly, a Goodpasture’s syndrome develops in genetically predisposed smokers, but exposure to hydrocarbons and respiratory viral infections are other possible triggers. The symptoms is to dyspnea, coughing, fatigue, hemoptysis, and hematuria. A Goodpasture’s syndrome is suspected in patients with hemoptysis or hematuria and detected by the detection of anti-GBM antibodies in the blood or kidney biopsy samples. The prognosis is good when treatment begins before developing a pulmonary or renal insufficiency. The therapy includes plasma exchange, corticosteroids and immunosuppressive agents such as cyclophosphamide. Pathophysiology a Goodpasture’s syndrome is the combination of glomerulonephritis with alveolar hemorrhage, and anti-GBM antibodies. Goodpasture’s syndrome is manifested most frequently as a combination of diffuse alveolar hemorrhage and glomerulonephritis, but as isolated glomerulonephritis (10-20%) or pulmonary disease may occasionally express (10%). Men are affected more frequently than women. Anti-GBM antibody directed against the non-collagenous (NC-1) domain of the ?3 chain of type IV collagen, which is found in very high concentrations in the basement membranes of the renal and pulmonary capillaries. By environmental factors – most commonly cigarette smoking, banal viral infections of the upper respiratory tract and inhalation of soluble hydrocarbons, rare pneumonia – alveolar capillary antigens are exposed to circulating antibodies in genetically predisposed, most often in the presence of HLA-DRw15-, -DR4- and DRB1 alleles , Circulating anti-GBM antibodies bind to the basement membrane, leading to Komplementablagerungen and trigger a cell-mediated inflammatory response that leads to glomerulonephritis and / or pulmonary Capillaritis. Symptoms and signs The most prominent symptom is hemoptysis. Nevertheless, they may be absent with alveolar hemorrhage and patients may have only infiltrates on chest x-ray or infiltrates associated with pulmonary insufficiency and / or failure in Patieten. Other common symptoms include dyspnea cough fatigue fever weight loss hematuria Up to 40% of patients have gross hematuria. However, pulmonary hemorrhage can manifest weeks to years prior to renal involvement. Alveolar hemorrhage (Goodpasture’s syndrome) with permission of the publisher. For Cohen A., R. Glassock In Atlas of Diseases of the Kidney: glomerulonephritis and vasculitis. Edited by R. Schrier (Series Editor), R.J. Glassock and A. H. Cohen. Philadelphia, Current Medicine, 1999. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/alveolar_hemorrhage_goodpastures_syndrome_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / ? images / alveolar_hemorrhage_goodpastures_syndrome_high_de.jpg lang = en & thn = 0 ‘, title:’ Alveolar hemorrhage (Goodpasture’s syndrome) ‘description:’ u003Ca id = “v37893137 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eBeidseitige alveolar opacities in a patient with alveolar hemorrhage due to Goodpasture’s syndrome u003c / p u003e u003c / div u003e ‘credits’. with approval the publisher. For Cohen A.

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