Liječnički vjesnik, Vol. 137 No. 5-6, 2015.
Stručni rad
GOODPASTURE’S SYNDROME – CASE REPORTS
Gordana Kotur
Goran Kotur
Ivica Horvatić
Danica Galešić Ljubanović
Krešimir Galešić
Sažetak
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 a-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
Ključne riječi
Anti-glomerular basement membrane disease – diagnosis, immunology, pathology, therapy; Pulmonary alveoli – pathology; Hemoptysis – etiology; Kidney glomerulus – pathology, immunology; Basement membrane – immunology; Autoantibodies – blood; Immunosuppressive agents – therapeutic use; Cyclophosphamide – therapeutic use; Plasmapheresis
Hrčak ID:
172704
URI
Datum izdavanja:
30.6.2015.
Posjeta: 2.807 *