Naslov
PAUCI IMMUNE RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS, CLINICAL AND HISTOPATHOLOGICAL DATA. EXPERIENCE FROM CROATIAN UNIVERSITY HOSPITAL
(SP166PAUCI IMMUNE RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS, CLINICAL AND HISTOPATHOLOGICAL DATA. EXPERIENCE FROM CROATIAN UNIVERSITY HOSPITAL)

Autori
Crnogorac, Matija ; Tisljar, Miroslav ; Ivica, Horvatic ; Toric, Luka ; Kacinari, Patricia ; Pehar, Mario ; Ljubanovic Galesic, Danica ; Galesic, Kresimir

Izvornik
Nephrology Dialysis Transplantation (0931-0509) 31 (2016), Suppl_1; I141-i141

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, ostalo

Ključne riječi
pauci immuneglomerulonephritis, ANCA,
(pauci immuneglomerulonephritis, ANCA)

Sažetak
Introduction and Aims: Retrospective cohort presents clinical and histopathological data of patients with pauci immune rapidly progressive glomerulonephritis (RPGN), diagnosed and treated in our hospital over 15 year period. (2000-2015.) Methods: 75 of 1517 patients, enrolled in our renal biopsy registry, with pauci immune RPGN were analysed. Based on patohistological and clinical data they were diagnosed with one of the small-vessel ANCA associated vasculitis: microscopic polyangiitis (MPA) 45, 3%, granulomatosis with polyangiitis (GPA) 22, 7%, eosinophilic granulomatosis with polyangiitis (EGPA) 4% or renal limited vasculitis (RLV) 28%. We analysed epidemiological data, blood pressure, extrarenal disease manifestations (respiratory, skin, general simptoms), serum creatinine (Sc), creatinine clearance (CrCl), 24h proteinuria, C-reactive protein (CRP), antineutrophilic cytoplasmic antibiodies (PR3 and MPO - ANCA), treatment, plasmapheresis (PF) and need for renal replacement therapy (RRT). Birmingham vasculitis activity score was calculated. Median value and interquartile(IQ) range of continuing variables were compared using Kruskal-Wallis and Mann-Whitney U test. Category variables were compared using χ2-test and Fisher Exact test. Analysis was performed using SPSS statistical software version 17.0. (p<0, 05) Results: 75 patients (52% female) median age 61 (IQ range 48-68). MPA patients were older than those with EGPA (p=0, 037) and RLV (p=0, 019), as to GPA there was no difference. Prevalence of general simptoms (88% of all patients) and arterial hypertension (52% of all patients) was not statistically different between entities. Most of the patients (48%) presented with rapidoprogressive nephritic syndrome (MPA 55, 99% ; GPA 82, 4%) while patients with RLV mostly presented with nephrotic syndrome (42, 9%). Respiratory symptoms were more associated with GPA (p=0, 004) and skin with nonGPA (p=0, 028) and nonRLV (p=0, 002). Majority of the patients had some degree of renal impairment with reduced CrCl (CKD-EPI formula) except for EGPA patients. MPA and GPA patients had significantley higher Sc and lower CrCl than those with EGPA (p=0, 026 for Sc ; 0, 017 for CrCl) and RLV (p=0, 003 ; 0, 001). There was no significant difference for 24h proteinuria. RLV patients had lower CRP compared to MPA (p=0, 01) and GPA (p=0, 021) patients. GPA patients had significantley higher BVAS compared to MPA (p=0, 019) and RLV (p=0, 006) patients. 17, 4% patients had PR3-ANCA, 36% MPO-ANCA, 5, 3% both ANCA subtipes and 41, 3% of the patients were ANCA negative. Analysing pathohistological types, MPA (47, 1%) and RLV(76, 2%) had significantly greater proportion of mixed type compared to others ( (p=0, 022), GPA presented predominantly as crescentic and EGPA as focal type. In majority of the patients (93, 4%) induction therapy included glucocorticoids and cyclophosphamide, 1, 3% glucocorticoid monotherapy and 5, 3% symptomatic treatment. 33, 3% of all patients were treated with PF and 32% of all patients required RRT. PF treatment was more associated with GPA (p=0, 011) and nonRLV (p=0, 001) and RRT with MPA (p=0, 04) and nonRLV (p=0, 009). Conclusions: Clinical and histological data on pauci-immune glomerulonephritis are basis for better understanding of these diseases and for determening prognostic factors. We also emphesize larger percentage of ANCA negative patients compared to other reports.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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