Volume 11 Supplement 1

7th Congress of International Society of Systemic Auto-Inflammatory Diseases (ISSAID)

Open Access

P02-028 - Muckle-Wells syndrome and renal transplantation

  • B Kortus-Götze1 and
  • J Hoyer1
Pediatric Rheumatology201311(Suppl 1):A135

https://doi.org/10.1186/1546-0096-11-S1-A135

Published: 8 November 2013

Introduction

The Muckle-Wells syndrome (MWS) is a rare inherited disease and belongs to the group of cryopyrin-associated periodic syndromes (CAPS). Recurrent fever attacks, myalgia, arthralgia, urticarial rash, headache, conjunctivitis, sensorineural deafness and a severe fatigue syndrome are the typical symptoms of MWS. Due to an unregulated production of IL1 a continuous formation of serum amyloid leads ultimately to the development of AA-amyloidosis, which is life-threatening and in some cases the fatal complication of MWS.

Case Report

Here we report the three years follow up on a 34-year old female patient with Muckle-Wells syndrome and biopsy proven systemic AA amyloidosis and end stage renal disease. After renal transplantation therapy with canakinumab subcutaneously in a dosage of 150 mg every eight weeks was continued in combination with the immunosuppressive therapy.

Discussion

Before and after renal transplantation the patient had a very good response to canakinumab with low activity in inflammation markers with an improved quality of life. Over the period of three years the triple immunosuppressive therapy (CSA, MMF, Prednisone) in combination with canakinumab has had no negative effect on activity of MWS and no pharmacological interactions between medication were observed. Even 3 years after renal transplantation, the patient remains an excellent kidney function without proteinuria. There are no signs of recurrence of AA-amyloidosis in the transplanted kidney.

Disclosure of interest

None declared.

Authors’ Affiliations

(1)
Nephrology, Internal Medicine, Nephrology

References

  1. According to our data, treatment with different immunomodulators in patients with Muckle-Wells syndrome and renal transplantation is safe, feasible and without severe side effects also over a longer time.Google Scholar

Copyright

© Kortus-Götze and Hoyer; licensee BioMed Central Ltd. 2013

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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