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CASE REPORT
Atypical haemolytic uremic syndrome (aHUS) and membranoproliferative glomerulonephritis (MPGN), different diseases or a spectrum of complement-mediated glomerular diseases?
  1. Ghada A Ankawi1,
  2. William F Clark2
  1. 1 London Health Sciences Centre, London, Ontario, Canada
  2. 2 Department of Medicine, Faculty of Medicine, Univ Western Ontario, London, Ontario, Canada
  1. Correspondence to Dr William F Clark, william.clark{at}lhsc.on.ca

Summary

Historically, patients with kidney diseases caused by genetic or acquired dysregulation of the complement alternative pathway have been grouped into clinical syndromes, C3 glomerulopathy (C3GN/DDD) and thrombotic microangiopathy (TMA), specifically atypical haemolytic uremic syndrome (aHUS). Recent data suggested that these diseases share a common pathophysiology and that patients can transition between glomerulopathies in this spectrum. Histopathologically, the main difference cited is the immunofluorescence (IF) findings, with C3 predominance in C3 glomerulopathy (compared with immunoglobulins and complements in immune complex-mediated membranoproliferative glomerulonephritis (MPGN)) and negative IF in TMA. We report a case in which a patient presented with hypertension, seizures, proteinuria, renal impairment and immune complex-mediated MPGN on kidney biopsy. Months later, she presented with classical TMA. She failed to respond to steroids and plasma exchange therapy but subsequently made a remarkable haematological and renal recovery after eculizumab treatment, thus supporting an underlying complement dysregulation and a diagnosis of aHUS.

  • renal intervention
  • acute renal failure

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Footnotes

  • Contributors Both authors contributed to the conception and design of the study and drafting the article. Both gave final approval of the version published. Both agreed to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved.

  • Competing interests WFC has received speaking honoraria from Octaplasma.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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