PMID: 9437406Jan 23, 1998Paper

Hypocomplementaemia caused by C3 nephritic factors (C3 NeF): clinical findings and the coincidence of C3 NeF type II with anti-C1q autoantibodies

Journal of Internal Medicine
L SkattumA G Sjöholm

Abstract

The main purposes were to document manifestations associated with prolonged or clinically unexplained C3 deficiency and to approximate how often hypocomplementaemia of this kind is caused by C3 nephritic factors (C3 NeF), i.e. autoantibodies to alternative pathway C3 convertases. We also wished to distinguish between C3 NeF types I and II and to assess coincident autoantibody responses to the collagen-like region of C1q (C1qCLR). The investigation was based on serum samples referred to a specialized laboratory for complement analysis in the course of several years. Twenty-five persons with C3 concentrations lower than 0.43 g L-1, a third of the normal, were included in the study. Analysis using three methods provided evidence of C3 NeF in 20 persons with equal frequencies of C3 NeF types I and II. We also gave evidence of antibody specificity differences for the two types of C3 NeF. Six patients with C3 NeF type II showed antibodies to C1qCLR. Membranoproliferative glomerulonephritis was the predominant diagnosis and two patients had partial lipodystrophy reflecting the well-known association between these diseases and C3 NeF. Anaphylactoid purpura, systemic lupus erythematosus, and severe infection, mainly meningococcal diseas...Continue Reading

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Citations

Oct 18, 2003·Metabolism: Clinical and Experimental·Zhunan Xia, Katherine Cianflone
Oct 12, 2010·Clinical Microbiology Reviews·Sanjay RamPeter A Rice
Jan 5, 2012·Bioinorganic Chemistry and Applications·Woei-Shyan LeeWen-Zhen Luo
Jan 26, 2016·Kidney International·Andrea AngioiAn S De Vriese
Aug 3, 2012·Kidney International·Danielle Paixão-CavalcanteClaire L Harris
Sep 6, 2015·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Edimara S ReisJohn D Lambris
Aug 1, 2008·Clinical Immunology : the Official Journal of the Clinical Immunology Society·L SkattumL Truedsson
Dec 12, 2012·Clinical & Developmental Immunology·Bo Nilsson, Kristina Nilsson Ekdahl
Oct 10, 2013·Virulence·Lisa A Lewis, Sanjay Ram
May 16, 2000·Plastic and Reconstructive Surgery·P J Guelinckx, N K Sinsel
Jul 28, 2016·World Journal of Nephrology·Maurizio Salvadori, Giuseppina Rosso
Mar 17, 2009·Pediatric Emergency Care·Andrew FretzayasMaria Moustaki
Jan 18, 2012·The Journal of Immunology : Official Journal of the American Association of Immunologists·Lisa SartzDiana Karpman
Nov 9, 2018·Frontiers in Immunology·Kristina N EkdahlBo Nilsson
Jan 25, 2003·Biochimica Et Biophysica Acta·Katherine CianfloneLan Ying Chen

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