title: [GOODPASTURE'S SYNDROME--CASE REPORTS].
abstract: Goodpasture's syndrome is a rare clinical entity characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and the presence of circulating autoantibodies to the glomerular basement membrane (GBM). Autoantibodies bind to reactive epitopes of noncollagenous domain of the collagen type IV alpha-3 chain in glomerular and alveolar basement membranes. Autoantibodies activate the complement cascade resulting in tissue injury by the type II hypersensitivity reaction according to the Coombs and Gell classification of antigen-antibody reactions. Prognostic factors include the renal excretory function and the degree of renal and lung damage at the time of presentation. Prompt diagnosis and early and adequate medical treatment is vital for patients. Clinical treatment must be aggressive in order of achieving better outcome. This article describes three patients who clinically presented with renopulmonary syndrome, renal failure, hematuria, proteinuria and hemoptysis. Kidney biopsy diagnosis was crescentic glomerulonephritis due to antibodies against GBM. In all three patients we started therapy with glucocorticoids and cyclophosphamide combined with plasma exchange therapy. In two patients who initially had severe impairment of renal function and high percentage of crescents in the renal biopsy, kidney function recovery was not achieved. In one patient, who at the time of clinical presentation showed milder renal failure and lower percentage of crescents in renal biopsy, the full recovery of renal function was obtained.
authors.forename: Gordana
authors.initials: G
authors.lastname: Kotur
authors.forename: Goran
authors.initials: G
authors.lastname: Kotur
authors.forename: Ivica
authors.initials: I
authors.lastname: Horvatić
authors.forename: Danica
authors.initials: D
authors.lastname: Galešić
authors.forename: L
authors.initials: L
authors.lastname: Jubanović
authors.forename: Krešimir
authors.initials: K
authors.lastname: Galešić
mesh_terms: D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D024141:Collagen Type IV / Q000276:immunology; D003520:Cyclophosphamide / Q000008:administration & dosage*; D018450:Disease Progression; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006469:Hemoptysis / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007677:Kidney Function Tests / Q000379:methods; D007678:Kidney Glomerulus / Q000473:pathology*; D008168:Lung / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000379:methods*; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D024141:Collagen Type IV; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; C057112:type IV collagen alpha3 chain; D003520:Cyclophosphamide