| Abstract | IgA nefropatija najčešći je primarni glomerulonefritis u svijetu, karakteriziran mezangijskim depozitima IgA uz varijabilnu kliničku prezentaciju i prognozu. Kod oko 30 % dolazi do postepene progresije bubrežne bolesti do završnog stadija. MEST-C zbroj u bioptatu klasificira prisutnost mezangijske i endokapilarne hipercelularnosti, segmentalne glomeruloskleroze, tubularne atrofije i intersticijske fibroze te prisutnosti polumjeseca. Godine 2019. je razvijen "Novi međunarodni alat za predviđanje rizika u IgA nefropatiji" koji na osnovi eGFR, krvnog tlaka i proteinurije pri biopsiji, dobi, rase, uporabe RAAS inhibitora u trenutku biopsije i MEST zbroja predviđa u maksimalnom razdoblju od 6,7 godina progresiju bubrežne bolesti do terminalnog stadija ili pad eGFR za 50 %. Nova verzija ovog alata omogućuje predviđanje rizika 1 ili 2 godine nakon biopsije. Aktualne KDIGO smjernice preporučuju primjenu kortikosteroida u trajanju 6 mjeseci samo u bolesnika koji imaju proteinuriju veću od 1 g/dU unatoč maksimalnoj tolerabilnoj dozi RAAS inhibitora u trajanju od minimalno 3 mjeseca, ostali su bolesnici kandidati za suportivno liječenje. Ostala imunosupresivna terapija nije preporučljiva. Cilj istraživanja je odrediti koji bolesnici imaju veću vjerojatnost da prime imunosupresivnu terapiju i koji su bubrežni ishodi bolesnika koji su liječeni imunosupresivnom terapijom. Proveli smo retrospektivno opservacijsko istraživanje, uključujući bolesnike sa novodijagnosticiranom idiopatskom IgA nefropatijom u razdoblju od 2012. do 2021. godine. Uključeno je 48 bolesnika (33 muškarca, srednje dobi 48±16 godina), s medijanom praćenja 37 mjeseci. Ukupno 18 (38%) bolesnika je primilo imunosupresivnu terapiju steroidima, ciklofosfamidom, MMFom, azatioprinom i oralnim budesnoidom. Bolesnici koji su liječni imunosupresivima su češće imali mezangijske lezije (M) (82 % naprema 54 %, p=0,05), endokapilarni hipercelularitet (E) (65 % naprema 21 %, p=0,004) i polumjesece (C) (41 % naprema 14 %, p=0,04). Nije bilo značajne razlike u eGFR kod biopsije (60±29 naprema 54±31 mL/min/1,73 m2, p<0.05), ali bolesnici liječeni imunosupresivima su imali bolji eGFR nakon 2 godine praćenja u usporedbi s onima koji su liječeni suportivnim mjerama (70±35 naprema 42±27 mL/min/1,73 m2, p=0,02). Proteinurija se snizila nakon liječenja (kod biopsije naprema kraju praćenja, 106 mg/mmol naprema 43 mg/mmol), ali nije bilo razlike u početnoj i proteinuriji na kraju praćenja kod onih koji jesu i koji nisu primali imunosupresive. IIgAN zbroj se smanjio nakon liječenja (pri biopsiji naprema nakon 2 godine praćenja, 10.56 %±12.66 % naprema 8.45 %±9.22 %, p=0,01), ali nije bilo razlike između onih koji jesu i koji nisu liječeni imunosupresivima. Zaključno, bolesnici sa višim M, E i C zbrojem su imali veću vjerojatnost biti liječeni imunosupresivima. Oni liječeni imunosupresivima imali su bolji eGFR nakon 2 godina, a na proteinurija na kraju praćenja i IIgAN zbroj su bili niži i kod bolesnika liječenih imunosupresivima i onih liječenih suportivno. |
| Abstract (english) | IgA nephropathy is the most common primary glomerulonephritis in the world, characterized by mesangial deposits of IgA with variable clinical presentation and prognosis. In about 30 %, there is a gradual progression of kidney disease to ESRD. The MEST-C score classifies the presence of mesangial and endocapillary hypercellularity, segmental glomerulosclerosis, tubular atrophy and interstitial fibrosis, and the presence of crescents in bioptic material. In 2019, the "New International Risk Prediction Tool in IgA Nephropathy" was developed, that based on eGFR, blood pressure and proteinuria at biopsy, age, race, RAAS inhibitor use at the time of biopsy and MEST score predicts progression of kidney disease to the terminal stage or a drop in eGFR by 50 % in time frame of 6.7 years. The update of this equation can be used to predict the risk at a
landmark time of 1 or 2 years after biopsy. Current KDIGO guidelines recommend the use of corticosteroids for 6 months only in patients who have proteinuria greater than 1 g/day despite the maximally tolerated dose of RAAS inhibitors for a minimum of 3 months, other patients are candidates for supportive treatment. Other immunosuppressive therapy is not recommended.
This study aimed to investigate which patients were more likely to receive immunosuppressive therapy and renal outcomes in patients treated with immunosuppressive therapy. We conducted a single-center retrospective observational study, including patients with newly diagnosed idiopathic IgAN from 2012 to 2021. We enrolled 48 patients (33 men, mean age 48±16 years), median follow-up of 37 months. A total of 18 (38%) patients received immunosuppressive therapy and were treated with steroids, cyclophosphamide, MMF, azathioprine and oral budesonide. Patients treated with immunosuppression had more frequently mesangial lesions (M) (82% vs. 54%, p=0.05), endocapillary hypercellularity (E) (65% vs. 21%, p=0.004) and crescents (C) (41% vs. 14%, p=0.04). There was no difference in eGFR at biopsy (60±29 vs. 54±31 mL/min/1.73 m2, p<0.05), but patients treated with immunosuppression had better eGFR after 2-year follow-up compared to those treated with supportive care (70±35 vs. 42±27 mL/min/1.73 m2, p=0.02). Proteinuria decreased after treatment (at biopsy vs. end of follow-up, 106 mg/mmol vs. 43 mg/mmol), but there was no difference in baseline or end-of-follow-up proteinuria between those receiving immunosuppression and not. IIgAN score decreased after treatment (at biopsy vs. at 2-year follow-up, 10.56%±12.66% vs. 8.45%±9.22%, p=0.01), but there was no difference in IIgAN score at biopsy or at 2-year follow-up between patients receiveing immunosuppression and those treated with non-immunosuppressive therapy. To conclude, patients with higher M, E and C score were more likely to be treated with immunosuppression. Those treated with immunosuppressants had better eGFR after 2 years, while proteinuria at the end of follow-up and IIgAN score improved with both supportive care and immunosuppression. |
