www.medenosrce.net/arhimed   arhimed(a-t>medenosrce.net   [22/04/2026 01:30:12] http://www.medenosrce.net/arhimed/poglej.asp?id=99 C1Q nefropatija: imunohistološke znacilnosti v primerjavi s histomorfološkimi spremembami Avtor: Mojca Rožic Mentor: znanst. sod. dr. Alenka Vizjak Somentor: akademik prof. dr. Dušan Ferluga Izhodišce: C1q nefropatija je opredeljena imunofluorescencno s prevladujocimi odlagami C1q v glomerulih pri bolnikih brez znakov za sistemski lupus eritematozus ali hipokomplementni urtikaricni vaskulitis. Opisi histomorfoloških sprememb v maloštevilnih raziskavah, ki zajemajo majhno število bolnikov, so raznoliki in neenotni. Ni bilo objavljene sistematicne primerjave med glomerulnimi histomorfološkimi spremembami in imunofluorescencnimi znacilnostmi glomerulnih imunskih odlag. Namen raziskave je bil 1. na velikem številu ledvicnih biopsij bolnikov s C1q nefropatijo sistematicno raziskati znacilnosti imunskih odlag, 2. primerjati znacilnosti imunskih odlag z glomerulnimi histomorfološkimi spremembami in 3. iz rezultatov sklepati na možne patogenetske mehanizme pri nastanku te glomerulne bolezni. Hipoteza: Raziskava naj bi potrdila hipotezo, da C1q nefropatija ni enotna klinicno-patološka entiteta. Metode: V retrospektivno raziskavo je bilo vkljucenih 67 bolnikov s C1q nefropatijo, od tega 23 žensk, 41 moških in 3 dajalci ledvice, za katere nismo imeli demografskih podatkov. Vzorci ledvicnega tkiva so bili v casu opravljene ledvicne biopsije obdelani s standardno svetlobno in imunofluorescencno mikroskopsko tehnologijo. Bolnike smo razdelili v 6 skupin glede na glomerulne histomorfološke spremembe. Po arhivskih fotografijah pozitivnih imunofluorescencnih izvidov smo ponovno ocenili obliko, sestav, intenzivnost in mesto odlaganja imunskih reaktantov v ledvicnem tkivu. Za statisticno vrednotenje rezultatov smo uporabili Kruskal-Wallisov test in Pearsonov c2 test. Rezultati: Najvec bolnikov s C1q nefropatijo je imelo idiopatski nefrotski sindrom (INS) z minimalnimi glomerulnimi spremembami oz. fokalno glomerulosklerozo (n=21), sledili so primeri s proliferacijskim in mešanim membranskim in proliferacijskim glomerulonefritisom (n=15), blagimi glomerulnimi mezangijskimi spremembami (n=13), normalno ledvico (n=10) in drugimi histološkimi spremembami (n=8). V vseh skupinah je bil po intenzivnosti vodec C1q, ki je bil najpogosteje srednje intenziven (3+). Odlage C1q so pogosto spremljali C3 (n=55), IgM (n=51), IgA (n=26), IgG (n=43) in manj pogosto C4 (n=17) in fibrin/fibrinogen (n=12). Najbolj pestre glomerulne imunske odlage, ki so jih pri 4 bolnikih spremljale tudi zunajglomerulne žilne in tubulo-intersticijske, smo ugotovili v skupini proliferacijskega glomerulonefritisa. Znacilno mesto odlaganja C1q in ostalih imunskih reaktantov je bil mezangij s pogosto izrazitejšimi odlagami v hilusu, le pri posameznih proliferacijskih glomerulonefritisih so bile odlage tudi v stenah glomerulnih kapilar. Zakljucki: Pri bolnikih s histološko normalno ledvico, z drugimi histološkimi spremembami in zelo verjetno tudi z INS z minimalnimi glomerulnimi spremembami oz. fokalno glomerulosklerozo, ki jih ne spremljajo glomerulne vnetne spremembe, so imunske odlage po vsej verjetnosti pasivne, posledica fiziološkega odstranjevanja imunskih kompleksov ali posledica zmanjšane sposobnosti fagocitoze mezangijskih celic. V skupini s proliferacijskim in mešanim membranskim in proliferacijskim glomerulonefritisom pa zrncaste imunske odlage zelo verjetno pomenijo odlaganje imunskih kompleksov. Glede na razlicne glomerulne histomorfološke vzorce, do neke mere razlicno klinicno sliko in možne razlicne patogenetske mehanizme menimo, da C1q nefropatija ni enotna klinicno-patološka entiteta. «» [Abstract / English version] C1q nefropathy: immunofluorescent characteristics compared with histomorfological changes Author: Mojca Rožic Mentor: znanst. sod. dr. Alenka Vizjak Co-mentor: akademik prof. dr. Dušan Ferluga Background: C1q nefropathy is defined by immunofluorescence with dominant glomerular C1q deposits in patients without evidence for systemic lupus erythematosus or hypocomplementemic urticarial vasculitis syndrome. Descriptions of histomorfological changes in few published studies including small number of patients are different and nonunified. No systematical comparison of glomerular histomorfological changes with immunofluorescent characteristics of glomerular immunological deposits has been published. The aim of this research work was 1. to evaluate the characteristics of immune deposits of a larger series of kidney biopsies patients with C1q nephropathy, 2. to compare the features of immune deposits with glomerular histomorphologic changes and 3. to draw conclusions from the results about the possible pathogenetic mechanisms involved in the developement of this glomerular disease. Hypothesis: We expect that this research work will confirm the hypothesis that C1q nefropathy is not a clinico-pathologic entity. Methods: This retrospective study included 67 patients with C1q nephropathy, among them 23 women, 41 men and three kidney donors, for which the demographic data were missing. Kidney biopsy tissue samples were at the time of biopsy examined by standard light and immunoflourescence microscopy techniques. The patients were according to the glomerular histomorphologic changes divided into 6 groups. Pattern, composition, intensity and the localisation of immune deposits in kidney tissue were reevaluated on the archive photographies of positive immunofluorescence results. The Kruskal-Wallis test and Pearsons c2 test were used for statistical evaluation of the results. Results: Patients with idiopathic nephrotic syndrome (INS) with minimal glomerular changes or focal glomerular sclerosis formed the largest group (n=21), followed by the group of patients with proliferative and mixed membraneous and proliferative glomerulonephritis (n=15), mild glomerular mesangial changes (n=13), histologically normal kidney (n=10) and the group of other histological changes (n=8). The C1q deposits were in all groups dominant or codominant and in most of the cases they showed the mean intensity (3+). The C1q deposits were frequently accompanied by C3 (n=55), IgM (n=51), IgG (n=43) and IgA (n=26) deposits and not so often with C4 (n=17) and fibrin/fibrinogen (n=12) deposits. The most variegated glomerular immune deposits, associated in four patients with extraglomerular capillary and tubulo-interstitial deposits were established in the group of proliferative and mixed membraneous and proliferative glomerulonephritis. The C1q deposits and other immune reactant deposits were typically located in the mesangium, frequentlly accentuated in the glomerular hilus. Only in some patients with proliferative glomerulonephritis the deposits were located in the wall of glomerular capillaries, too. Conclusions: Immune deposits that are not accompanied by glomerular inflammatory reaction in patients with histologically normal kidney, other histological changes and probably also in patients with INS with minimal glomerular changes or focal glomerular sclerosis are most probably a consequence of passive entrapment of the immunological reactants, physiological removal of immune complexes or reduced phagocytic capacity of mesangial cells. The granular deposits in patients with proliferative and mixed membraneous and proliferative glomerulonephritis most probably suggest for immune complex deposition. According to different histomorphologic patterns, somehow different clinical picture and very likely different pathogenetic mechanisms, we assume that C1q nephropathy is not a distinct clinico-pathologic entity.