Naslov
Cellular variant of focal segmental glomerulosclerosis – a single centre experience

Autori
Kasumović, Dino ; Torić, Luka ; Zagorec, Nikola ; Horaček, Matija ; Tišljar, Miroslav ; Šenjug, Petar ; Galešić Ljubanović, Danica ; Galešić, Krešimir

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, ostalo

Skup
56th ERA-EDTA Congress

Mjesto i datum
Budimpešta, Mađarska, 13.06.2019. - 16.06.2019

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
focal segmental glomerulosclerosis, cellular variant, outcome

Sažetak
INTRODUCTION: Focal segmental glomerulosclerosis (FSGS) is one of the most prevalent glomerulopathies in the world. According to the Columbia classification that relies on specific histopathological findings there are five variants of FSGS: collapsing, tip, cellular, perihilar and not otherwise specified (NOS). In majority of studies NOS variant is the most prevalent one, and cellular is often one of the rarest. Cellular variant is defined by finding of at least one glomerulus with segmental endocapillary hypercellularity occluding lumina with or without foam cells and karyorrhexis. Histopathological variants of FSGS can correlate with prognosis of the disease. Our aim was to investigate the cellular variant, its clinical characteristics and outcomes in patients that were treated in our hospital. METHODS: We investigated clinical and histopathological data from all patients with cellular FSGS who were diagnosed with the disease between years 2000 and 2016 at our hospital. We collected data using our Renal biopsy register. Following definitions were used for the purpose of outcome analysis. Complete remission (CR) was defined as a reduction in proteinuria to <0.3 g/dU with normal kidney function and partial remission (PR) was defined as a reduction in proteinuria by >50% from the initial value or 0.3-3.5g/dU proteinuria with stable kidney function (no more than a 20% increase in serum creatinine). Relapse was defined as proteinuria ≥3.5 g/dU after complete remission has been obtained. End-stage kidney disease (ESKD) was defined as a persistent decrease in eGFR under 15 ml/min/1.73m2 or beginning of renal replacement therapy and persistent kidney dysfunction (PKD) was defined as a failure to meet criteria for remission but not reaching ESKD. RESULTS: Out of 180 FSGS patients, 11 (6.1%) had a cellular variant. The mean age at the time of renal biopsy was 51 years, there were 8 males and 3 females. All patients presented with nephrotic proteinuria. The 24-hour proteinuria ranged from 4.7 to 47.8g/dU and 8 patients had massive proteinuria (>10g/dU). Eight patients had concomitant microhematuria, 3 patients had high levels of creatinine (>200µmol/L) and 7 patients had arterial hypertension. The level of tubulointerstitial damage was very heterogeneous. All cases were diagnosed as primary FSGS and all had >50% podocyte foot process effacement on electron microscopy. All patients were treated with renin-angiotensine-aldosterone system blockade. At dismission 7 patients were treated with corticosteroid therapy alone and 4 were treated with combination of corticosteroid and cyclosporin. Three patients were lost to regular follow up and two had the last control before one year after initiation of treatment. Of the remaining patients (6) after 1 year of treatment one had CR and one had PR, there was no remission in other four. After the last follow up (average follow up time was 6 years) 3 patients reached CR and 3 were still without remission having a PKD because of the fall in eGFR and not because of the proteinuria. No one reached ESKD. CONCLUSIONS: Cellular variant of FSGS is a rare glomerulopathy and a histopathological diagnosis of cellular FSGS can be challenging. Although a lot of our patients presented even with massive proteinuria they generally responded good to the immunosuppressive treatment in terms of reduction of proteinuria. Because it is a rare condition larger studies with patients from different centres might bring more information.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA