Goodpasture syndrome

master's thesis

• Title (croatian): Goodpasturov sindrom
• Author: Guy Alush
• Mentor: Živka Dika (mentor)
• Committee member: Živka Dika (predsjednik povjerenstva)
• Committee member: Nikolina Bašić Jukić (član povjerenstva)
• Committee member: Ivana Vuković Brinar (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; Zagreb
• Defense date and country: 2023-07-13, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine

Abstract

This graduation thesis is a narrative review for Goodpasture syndrome (GS), a rare autoimmune disease affecting the kidneys and lungs. This eponymous syndrome, first described by Goodpasture in 1919, refers to the renopulmonary vascular damage done by circulating antibodies and their subsequent post-deposition inflammatory damage. The complex pathogenesis of the disease includes genetic predisposition, central tolerance defect, glomerular basement membrane (GBM) exposure revealing a culprit epitope, subsequent antibodies production against GBM and finally, culmination with antibody deposition in the kidneys and lungs. The autoantibodies thereafter, trigger a fulminant inflammatory reaction carried by cell-mediated and complement-dependent immune processes. The symptoms that follow range in severity from dyspnea to pulmonary hemorrhage and respiratory failure in the lungs, and from oliguria and hematuria to rapidly progressive glomerulonephritis (RPGN) in the kidneys. Therefore, physicians should have a high clinical suspicion of GS with any respiratory symptoms presentation in RPGN patients. Early diagnosis is imperative for treatment and is performed through serological tests and kidney biopsy. Treatment revolves around antibodies removal through plasma exchange and immunosuppressive therapy to halt further antibodies production. Pulmonary symptoms usually resolve, while recovery of renal function is poor and patients often require renal replacement therapy. Fortunately, GS seldom recur and patients who survive the initial ailment have a favorable prognosis.

Abstract (croatian)

Ovaj diplomski rad narativni je prikaz Goodpastureovog sindroma (GS), rijetke autoimune bolesti koja zahvaća bubrege i pluća. Ovaj istoimeni sindrom, koji je prvi opisao Goodpasture 1919 godine, odnosi se na renopulmonalno vaskularno oštećenje uzrokovano cirkulirajućim protutijelima s posljedičnim upalnim oštećenjem nakon odlaganja istih. Složena patogeneza bolesti uključuje genetsku predispoziciju, poremećaj centralne tolerancije, izlaganje bazalne membrane (GBM) otkrivajući pri tome ključni epitop, potom proizvodnju protutijela protiv GBM-a i konačno, kulminaciju s taloženjem protutijela u bubrezima i plućima. Protutijela zatim pokreću fulminantnu upalnu reakciju posredovanu staničnim imunološkim procesima i komplementom. Simptomi koji slijede variraju u težini od dispneje do plućnog krvarenja i respiratornog zatajenja u plućima, te od oligurije i hematurije do brzo progredirajućeg glomerulonefritisa (RPGN) u bubrezima. Stoga bi liječnik trebao posumnjati na GS kod pojave bilo kakvih respiratornih simptoma u bolesnika s RPGN-om. Rana dijagnoza je imperativ za liječenje, a provodi se serološkim testovima i biopsijom bubrega. Liječenje je usmjereno na uklanjanje protutijela putem izmjene plazme i imunosupresivnom terapijom kako bi se zaustavila daljnja proizvodnja protutijela. Plućni simptomi obično nestaju, dok je oporavak bubrežne funkcije slab i pacijentima je često potrebno bubrežno nadomjesno liječenje. Srećom, GS se rijetko ponavlja i pacijenti koji prežive početnu epizodu bolesti imaju povoljnu prognozu.

• Keywords: Goodpasture syndrome; kidneys; lungs
• Keywords (croatian): Goodpasturov sindrom; bubrezi; pluća
• Language: english
• URN:NBN: urn:nbn:hr:105:531951
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2023-08-30 10:47:21