Goodpastureov sindrom – prikazi bolesnika [Goodpasture's syndrome - case reports]

Kotur, Gordana and Kotur, Goran and Horvatić, Ivica and Galešić Ljubanović, Danica and Galešić, Krešimir (2015) Goodpastureov sindrom – prikazi bolesnika [Goodpasture's syndrome - case reports]. Liječnički vjesnik, 137 (5-6). pp. 171-176. ISSN 0024-3477

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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.

Abstract in Croatian

Goodpastureov sindrom rijedak je klinički entitet karakteriziran brzoprogresivnim glomerulonefritisom (BPGN), difuznim plućnim hemoragijama i prisutnošću cirkulirajućih autoprotutijela na glomerularnu bazalnu membranu (GBM). Autoprotutijela se vežu na reaktivne epitope nekolagene domene a-3 lanca kolagena tipa IV, sastavnog dijela alveolarne i glomerularne bazalne membrane, te aktiviraju kaskadu komplementa, što rezultira oštećenjem tkiva po tipu II reakcije preosjetljivosti prema Coombsu i Gellu. Prognostički faktori uključuju patohistološke promjene i promjene ekskrecijske funkcije bubrega te stupanj plućnog oštećenja u trenutku očitovanja bolesti. Brzo postavljena dijagnoza i što ranije provedeno adekvatno liječenje osobito su važni za bolesnike. Agresivno liječenje kombinacijom imunosupresivne terapije i plazmafereze ima najbolje izglede za uspjeh. U ovom radu prikazano je troje bolesnika koji su se klinički očitovali renopulmonalnim sindromom: bubrežnim zatajenjem, hematurijom, proteinurijom i hemoptizama. Histološki u tkivu bubrega dobivenom biopsijom u svih bolesnika radilo se o glomerulonefritisu s polumjesecima uzrokovanom protutijelima na GBM. U sve troje bolesnika provedeno je liječenje glukokortikoidima, ciklofosfamidom i plazmaferezom. Svi bolesnici preživjeli su uz remisiju plućne bolesti. U dva bolesnika koja su u trenutku kliničke prezentacije imala znatno sniženu ekskrecijsku bubrežnu funkciju i visok postotak polumjeseca u tkivu bubrega dobivenog biopsijom došlo je do trajnoga gubitka bubrežne funkcije. U jednog bolesnika koji je u trenutku očitovanja bolesti pokazivao lakše bubrežno oštećenje te manji postotak polumjeseca došlo je do potpunog oporavka bubrežne funkcije.

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