Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome.

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Abstract

Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of chronic nephropathy recently reclassified into immunoglobulin-associated MPGN (Ig-MPGN) and C3 glomerulopathy (C3G). In this study we aimed: (1) to evaluate the complement genetic and biochemical profile in patients with Ig-MPGN/C3G; (2) to investigate whether genetic variants and different patterns of complement activation (i.e., fluid versus solid phase) correlate with disease manifestations and outcomes.

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Journal ID (iso-abbrev): Mol. Immunol.

Title: Molecular immunology

Publisher: Elsevier BV

ISSN (Electronic): 1872-9142

ISSN (Print): 0161-5890

Publication date (Electronic): Mar 2016

Volume: 71

Affiliations

[1 ] IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Ranica, Bergamo, Italy.

[2 ] Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy.

[3 ] Unit of Pediatric Nephrology, Dialysis and Transplantation, Azienda Ospedaliera of Padova, Italy.

[4 ] Department of Immunology, Assistance Publique-Hopitaux de Paris, Hopital Europeen George-Pompidou and INSERM UMRS 1138, Cordelier Research Center, Complement and Diseases Team, Paris, France.

[5 ] Division of Nephrology and Dialysis, Bambin Gesù Pediatric Hospital, Roma, Italy.

[6 ] IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Ranica, Bergamo, Italy. Electronic address: erica.daina@marionegri.it.

[7 ] IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Ranica, Bergamo, Italy; Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy; Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy.

Article

Publisher Item ID: S0161-5890(16)30011-6

DOI: 10.1016/j.molimm.2016.01.010

PubMed ID: 26895476

SO-VID: 26c5cf4e-d35e-4c22-90c5-07b467dc6c10

History

Keywords: C3 glomerulonephritis,C3 glomerulopathy,Dense-Deposit Disease,Membranoproliferative glomerulonephritis,Rare diseases

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Keywords: C3 glomerulonephritis, C3 glomerulopathy, Dense-Deposit Disease, Membranoproliferative glomerulonephritis, Rare diseases

Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome.

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