Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature

Lucio Manenti, Elisa Gnappi, Augusto Vaglio, Landino Allegri, Marina Noris, Elena Bresin, Francesco Paolo Pilato, Elisabetta Valoti, Sonia Pasquali, Carlo Buzio

Research output: Contribution to journalArticlepeer-review

Abstract

Background. Primary or secondary glomerulonephritis has been anecdotally reported in association with atypical haemolytic uraemic syndrome (aHUS). We here report a series of six patients who developed aHUS and glomerulopathy, and review the literature on aHUS and glomerulonephritis. Methods. Out of all patients diagnosed at our unit with biopsy-proven glomerular diseases between March 2007 and October 2011, selected cases developing aHUS during the follow-up are presented. The following tests were performed in all six patients: serum C3 and C4 levels, ADAMTS13 activity, CFH levels and anti-CFH autoantibodies and genetic screening for CFH, MCP, CFI, C3 and CFHR1-3 mutations and risk haplotypes associated with aHUS. Results. Two hundred and forty-eight patients received a biopsy-proven diagnosis of glomerulopathy and were followed for a median of 31 months (range 2-58). Of these, six developed aHUS, within a median of 15 months (range 1-36) of their initial diagnosis of glomerulopathy. One of these patients had focal segmental glomerulosclerosis (FSGS), two membranoproliferative glomerulonephritis (MPGN) type I, one C3 glomerulonephritis and two systemic small vessel vasculitis [one granulomatosis with polyangiitis (Wegener's), one Henoch-Schoenlein purpura]. Five patients (one of them heterozygous for a CFH mutation) carried, in homo- or heterozygosity, the risk haplotype CFH-H3 (CFH tgtgt), previously described to be associated with aHUS, while another one patient was homozygous for the MCPggaac risk haplotype predisposing to aHUS when present on both alleles. Conclusions. Different types of glomerulopathies can be complicated by aHUS. Several mechanisms can contribute to this association, such as nephrotic-range proteinuria, mutations or aHUS-risk haplotypes involving genes encoding alternative complement regulatory proteins in some patients and inflammatory triggers associated with systemic immune-mediated diseases.

Original languageEnglish
Pages (from-to)2246-2259
Number of pages14
JournalNephrology Dialysis Transplantation
Volume28
Issue number9
DOIs
Publication statusPublished - Sep 2013

Keywords

  • Atypical haemolytic uraemic syndrome
  • Glomerulonephritis
  • Nephrotic syndrome
  • Thrombotic microangiopathy
  • Vasculitis

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

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