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Pregled bibliografske jedinice broj: 853634

Zbornik radova

Autori: Gardijan, Bojana; Galešić Ljubanović, Danica; Jureković, Željka; Cingel, Branislav; Knotek, Mladen
Naslov: A BPI-ANCA Associated Rapidly Progressive Glomerulonephritis with Immune Complexes: A Case Report
( A BPI-ANCA Associated Rapidly Progressive Glomerulonephritis with Immune Complexes: A Case Report )
Izvornik: Journal of the American Society of Nephrology
ISSN: 1046-6673
Skup: American Society of Nephrology Kidney Week 2016
Mjesto i datum: Chicago, SAD, 15.-20.11.2016
Ključne riječi: BPI-ANCA; Rapidly Progressive Glomerulonephritis; Immune Complexes
( 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.
Rad je indeksiran u
bazama podataka:
Current Contents Connect (CCC)
MEDLINE
Scopus
Science Citation Index Expanded (SCI-EXP) (sastavni dio Web of Science Core Collectiona)
Vrsta sudjelovanja: Poster
Vrsta prezentacije u zborniku: Sažetak
Vrsta recenzije: Međunarodna recenzija
Projekt / tema: 198-0000000-3355
Izvorni jezik: eng
Kategorija: Stručni
Znanstvena područja:
Kliničke medicinske znanosti
Upisao u CROSBI: Danica Ljubanović (danica.ljubanovic@zg.t-com.hr), 3. Sij. 2017. u 21:45 sati



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