IgA-vaskulitis sa zahvaćanjem bubrega u odrasloj dobi: iskustva iz Kliničke bolnice Dubrava, Za greb

Ana Gudelj Gračanin, Tea Mikula, Ivica Horvatić, Luka Torić, Majda Golob, Jasminka Dobša, Matea Liskij, Gabrijela Buljan, Karla Draženović, Matej Nedić, Danica Galešić Ljubanović, Krešimir Galešić

Introduction: IgA vasculitis, formerly named Henoch-Schönlein purpura, is a systemic small-vessel vasculitis that occurs commonly in children, while in adults, the disease remains rare. It is characterised by non-thrombocytopenic palpable purpura, arthritis, arthralgia, kidney disease and gastrointestinal pain. This study aimed to determine clinical signs and symptoms, laboratory and pathohistological parameters and therapy of IgA vasculitis in adults with renal involvement, by conducting our research on a sample of patients of a tertiary referral hospital. Research subjects and methods: In this retrospective cohort study, patients treated at Clinical Hospital Dubrava from 2011 to 2019 were included, who were diagnosed with IgA vasculitis, based on clinical, laboratory and pathohistological criteria. Clinical and laboratory parameters, were determined through the use of methods commonly used in
clinical practice. In patients with symptoms and signs of kidney involvement and after patients’ consent, kidney biopsy was performed, and analysed by light, immunofluorescence and electron microscopy. Results: twenty-two adults (12 M, 10 F), whose median age was 57, were included in our study. Palpable purpura and nephritis were present in all patients. Thirteen (59.1%) patients had arthralgia and five of them (22.5%) experienced abdominal pain. Respiratory infection preceded vasculitis in six (27.3%) patients, six patients ((27.3%) were taking medications and got vaccinated prior to the occurrence of vasculitis, and in ten (45.4%) patients the predisposing factor was unknown. Asymptomatic hematuria was present in ten (47.6%), subnephrotic proteinuria in three
(14.3%), nephrotic syndrome in nine (42.9%) and nephritic syndrome in three (14.3%) patients. According to the findings of kidney biopsy, which was performed on 18 patients, eleven (61.6%) had mesangial proliferation, fourteen (77.8%) had endocapillary proliferation, and eleven (61.6%) had extracapillary (crescents) proliferation. After the diagnosis, renin-angiotensin inhibitors were used by eighteen (81.8%) patients, glucocorticoids by twenty-one (95.5%) and cyclophosphamide by five (22.7%) patients. Patient follow-up was performed for 23 to 84 months and, in the said period, all of them had improved or stagnant renal function, except for one patient who died. Conclusion: Renal manifestations of IgA vasculitis may change the commonly mild course of this disease and lead to an increase in morbidity as well as affect the type and duration of treatment. So, in everyday work, it is important to determine characteristics of renal changes in IgA vasculitis in order for an adequate therapeutic option to be applied.

Uvod: IgA-vaskulitis, prije nazivan Henoch-Schönleinova purpura, vaskulitis je malih krvnih žila posredovan imunoglobulinom A (IgA). Najčešće obolijevaju djeca, dok je u odraslih incidencija manja. Kliničkom slikom dominiraju palpabilna, netrombocitopenična purpura, artralgije, artritis, znakovi bubrežne bolesti i abdominalna bol. Cilj ovog istraživanja bio je utvrđivanje kliničkih i laboratorijskih karakteristika te načina liječenja odraslih oboljelih od IgA-vaskulitisa sa zahvaćanjem bubrega na uzorku bolesnika iz jednoga tercijarnog centra. Ispitanici i metode: U retrospektivno kohortno istraživanje uključeni su bolesnici liječeni u razdoblju od 2011. do 2019. godine u Kliničkoj bolnici Dubrava u Zagrebu, kojima je na osnovi kliničkih, laboratorijskih i patohistoloških kriterija dijagnosticiran IgA-vaskulitis. Klinički i laboratorijski parametri određivani su metodama uobičajenima u kliničkoj praksi. U bolesnika sa znakovima zahvaćanja bubrega učinjene su biopsija bubrega i analiza svjetlosnom, imunofluorescentnom i elektronskom mikroskopijom. Rezultati: U istraživanju su sudjelovala ukupno 22 bolesnika (12 muškaraca, 10 žena), medijana dobi 57 godina. Svi su bolesnici imali purpuru i znakove bubrežne bolesti. Artralgije je imalo 13-ero (59,1%) bolesnika, a bol u trbuhu njih 5-ero (22,5%). Infekcija dišnog sustava prethodila je vaskulitisu u 6-ero (27,3%) bolesnika, lijekovi i cjepiva također u njih 6-ero (27,3%), a u 10-ero (45,4%) bolesnika predisponirajući čimbenik bio je nepoznat. Asimptomatsku hematuriju imalo je 10-ero (47,6%) bolesnika, subnefrotsku proteinuriju njih troje (14,3%), nefrotski sindrom 9-ero (42,9%), a nefritički sindrom imalo je također troje (14,3%) bolesnika. Prema nalazu biopsije bubrega, koja je učinjena u 18-ero bolesnika, mezangijsku proliferaciju imalo je njih 11-ero (61,1%), endokapilarnu proliferaciju 14-ero (77,8%), a ekstrakapilarnu proliferaciju (polumjesece) imalo je 11-ero (61,1%) bolesnika. Nakon postavljanja dijagnoze terapiju inhibitorima sustava renin-angiotenzin primalo je 18-ero (81,8%) bolesnika, 21 bolesnik (95,5%) dobivao je glukokortikoide, a njih 5-ero (22,7%) liječeno je ciklofosfamidom. Bolesnici su praćeni tijekom razdoblja od 23 do 84 mjeseca, a u svih se, osim u jedne bolesnice, bubrežna funkcija poboljšala ili je stagnirala. Zaključak: Bubrežne manifestacije IgA-vaskulitisa mijenjaju inače blagi tijek bolesti, dovode do porasta morbiditeta i utječu na trajanje liječenja. Pri svakodnevnom radu valja utvrditi značajke bubrežnih promjena kod IgA-vaskulitisa da bismo na temelju tih obilježja primijenili prikladnu terapiju.