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Kidney transplantation as a treatment of choice for AA amyloidosis due to periodic fever syndrome

Clinical Rheumatology volume 40, pages 763–768 (2021)Cite this article

Abstract

Renal AA amyloidosis is the most serious complication of periodic fever syndrome, which, inadequate suppression, due to persistent inflammation, leads to nephrotic syndrome and renal failure over several years. In most cases, periodic fever syndromes begin to manifest clinically in early childhood. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation (KT) is an effective and safe treatment for end-stage chronic kidney disease (CKD) based on AA amyloidosis. In this paper, we present cases of two patients after deceased donor KT, who have been diagnosed with adult periodic fever syndrome. In the first one, diagnosis and treatment began in advanced stage of CKD and therefore underwent KT with compensated disease, while in the second patient, the disease manifested and diagnosed in the post-KT period. Timely initiation of treatment ensured protection of the graft from amyloid deposition.

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Affiliations

  1. Department of Surgery and Transplantation Center, University Hospital Martin and Jessenius Medical Faculty of Comenius University, Kollárova 2, 03601, Martin, Slovak Republic

    K. Graňák, Matej Vnučák, P. Skálová, Ľ. Laca & I. Dedinská

  2. Department of Children and Adolescents, University Hospital Martin and Jessenius Medical Faculty of Comenius University, Kollárova 2, 03601, Martin, Slovak Republic

    M. Jeseňák

  3. 1st Department of Internal Diseases, University Hospital Martin and Jessenius Medical Faculty of Comenius University, Kollárova 2, 03601, Martin, Slovak Republic

    M. Mokáň

Authors
  1. K. Graňák
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  2. M. Jeseňák
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  3. Matej Vnučák
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  4. P. Skálová
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  5. Ľ. Laca
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  6. M. Mokáň
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  7. I. Dedinská
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Contributions

MUDr. Karol Graňák: participated in performing the research and writing the paper

Prof. MUDr. Miloš Jeseňák, PhD., MBA, Dott. Ric., MHA: participated performing research

MUDr. Matej Vnučák, PhD.: participated in writing the paper

MUDr. Petra Skálová: participated performing research

Prof. MUDr. Ľudovít Laca, PhD.: participated in writing the paper

Prof. MUDr. Marián Mokáň, DrSc, FRCP Edin: participated in performing research

Doc. MUDr. Ivana Dedinská, PhD.: participated in writing the paper

Corresponding author

Correspondence to Matej Vnučák.

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The authors declare no conflicts of interest. The authors declare no funding was received for this study. Patients in submitting case reports have given written informed consent for publishing.

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Graňák, K., Jeseňák, M., Vnučák, M. et al. Kidney transplantation as a treatment of choice for AA amyloidosis due to periodic fever syndrome. Clin Rheumatol 40, 763–768 (2021). https://doi.org/10.1007/s10067-020-05283-1

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  • DOI: https://doi.org/10.1007/s10067-020-05283-1

Keywords

  • Autoinflammatory disease
  • Kidney transplantation
  • Periodic fever syndrome
  • Renal amyloidosis