Naslov
A BPI-ANCA Associated Rapidly Progressive Glomerulonephritis with Immune Complexes: A Case Report

Autori
Gardijan, Bojana ; Galešić Ljubanović, Danica ; Jureković, Željka ; Cingel, Branislav ; Knotek, Mladen

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

Izvornik
Journal of the American Society of Nephrology / - , 2016

Skup
American Society of Nephrology Kidney Week 2016

Mjesto i datum
Chicago (IL), Sjedinjene Američke Države, 15.11.2016. - 20.11.2016

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
BPI-ANCA; Rapidly Progressive Glomerulonephritis; Immune Complexes

Sažetak
Introduction: Antineutrophil cytoplasmic antibodies are a major cause of rapidly progressive glomerulonephritis (RPGN). Routinely tested ANCA are MPO and PR3. A number of atypical ANCA is also known, such as anti- bactericidal/permeability-increasing protein(BPI) ANCA, which is a well-described cause of vasculitis. Overlap syndromes of pauci-immune ANCA GN and immune complexes (IC)- mediated GN have been reported. After a review of literature, this is the first reported case of BPI-ANCA associated RPGN with IC. Case Description: A 35- year-old Caucasian male with cryptogenic liver cirrhosis was referred to our hospital with the diagnosis of hepatorenal syndrome. At admission, the pt was dialysis-dependent and oliguric. An active urine sediment (E >50, L 15, casts) was noted with P/C 2.6 (g/g). Kidney biopsy was performed. The finding was diffuse endocapillary proliferative glomerulonephritis, active, without signs of chronicity. Immunoflorescence (IF) was positive for IgG, IgM, C3, k and l chains. ANA, ENA, dsDNA, RF, anti-CCP, LAC, cardiolipin IgG and IgM, beta-2 glycoprotein were negative. Serum C3 was 0.53 g/L (0.90-1.80) and C4 0.15g/l (0.10- 0.40). Testing for HCV, HBV and HIV was negative, as well as bacterial respiratory and urine cultures. ANCA was positive by IF (1:640), with negative ELISA for PR3- and MPO-ANCA. ANCA was specified as BPI-ANCA. The pt was treated with combination of PEX, steroid pulses and iv cyclophosphamide (CYC). After 4 PEX, ANCA declined to 1:160. After 10 days the pt was no longer dialysis-dependent. He received in total 6 CYC pulses. After a follow-up of 6 months, his eGFR is 115ml/ min/1.73m2, without pathological proteinuria. Discussion: This is to our knowledge the first described case of BPI-ANCA-associated RPGN with IC. Pathohistology corresponded to lupus grade IVa nephritis (lupus-like nephritis, as serology was negative). Due to ANCA, PEX was added to steroids and CYC. This is an educational case demonstrating the presence of BPI-ANCA in a pt with IC GN. ANCA testing by IF is important for detection of atypical ANCA.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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