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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}


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)
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  • 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.

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