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Case Reports: Rare disease
Eculizumab therapy and complement regulation in a case of resistant catastrophic antiphospholipid syndrome
  1. Rachel Frederick1,2,
  2. Luigi Zolio3,
  3. Evange Romas3,4 and
  4. Frank Ierino1,4
  1. 1Department of Nephrology, St Vincent's Hospital Melbourne Pty Ltd, Fitzroy, Victoria, Australia
  2. 2Department of General Medicine, St Vincent's Hospital Melbourne Pty Ltd, Fitzroy, Victoria, Australia
  3. 3Department of Rheumatology, St Vincent's Hospital Melbourne Pty Ltd, Fitzroy, Victoria, Australia
  4. 4The University of Melbourne, Melbourne, Victoria, Australia
  1. Correspondence to Dr Rachel Frederick; rachfred99{at}gmail.com

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening form of antiphospholipid syndrome characterised by diffuse arterial and venous thrombosis, in the presence of positive antiphospholipid antibodies. The multiple sites of thrombosis in small, medium and large vessels progress to multiorgan failure, accounting for the high mortality rate associated with CAPS. Unregulated complement activation is increasingly recognised as critical to the pathogenesis of CAPS. Early diagnosis is essential to initiate prompt life-saving treatment with the triple therapy of anticoagulation, immunosuppression and either plasmapheresis or intravenous immunoglobulin. Among other immunosuppressive agents, eculizumab, a complement inhibitor has demonstrated efficacy in treatment-resistant cases.

We report an instructive case of a woman presenting with both clinical and laboratory findings consistent with primary CAPS, resistant to initial treatment and responsive to eculizumab, with emphasis on genetic testing and implications for future therapy.

  • Acute renal failure
  • Renal medicine
  • Renal intervention
  • Medical management

https://doi.org/10.1136/bcr-2022-254449

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    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: RF, LZ, ER and FI. The following authors gave final approval of the manuscript: RF, LZ, ER and FI.

    • 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.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

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