Article Text

Download PDFPDF
CASE REPORT
Acquired C1-inhibitor deficiency presenting with nephrotic syndrome
  1. Jamie Willows1,
  2. Katrina Wood2,
  3. Helen Bourne3 and
  4. John Andrew Sayer1,4
  1. 1 Renal Services, Freeman Hospital, Newcastle upon Tyne, UK
  2. 2 Histopathology Department, Royal Victoria Infirmary, Newcastle upon Tyne, UK
  3. 3 Immunology Department, Freeman Hospital, Newcastle upon Tyne, UK
  4. 4 Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK
  1. Correspondence to Professor John Andrew Sayer, john.sayer{at}ncl.ac.uk

Abstract

Acquired C1-inhibitor (C1-INH) deficiency is a rare and potentially life-threatening disorder, which presents with recurrent attacks of non-pitting oedema to the face, airway, limbs or gastrointestinal tract. It is often associated with underlying B-cell lymphoproliferative disorders. We describe a case of a 73-year-old man with acquired C1-INH deficiency who presented with nephrotic syndrome due to glomerular IgM deposition, secondary to an underlying secretory lymphoplasmacytic lymphoma. Both the acquired C1-INH deficiency and the nephrotic syndrome resolved when the underlying B-cell lymphoma was treated with rituximab and bendamustine, suggesting the underlying lymphoproliferative malignancy was driving both disorders.

  • renal system
  • immunology
  • haematology (incl blood transfusion)
View Full Text

Statistics from Altmetric.com

Footnotes

  • Contributors JAS conceived the study. JW and JAS wrote the initial draft of the paper. KW and HB contributed data and revised the paper. JAS finalised the paper.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

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

  • Patient consent for publication Obtained.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.