FAMILIAL MEDITERRANEAN FEVER COMPLICATED BY CHRONIC KIDNEY DISEASE STAGE 5 DESCRIPTION OF THE CLINICAL CASE

  • Maria Palchukovska Bogomolets National Medical University, Kyiv, Ukraine
  • Lyudmila Liksunova Candidate of Medical Sciences, Chief Physician of the Institute of Nephrology of the National Academy of Medical Sciences of Ukraine
Keywords: Amyloidosis, familial mediterranean fever, nephrotic syndrome, renal insufficiency, peritoneal dialysis

Abstract

 this clinical case demonstrates renal amyloidosis – the most severe complication of familial Mediterranean fever (FMF). This clinical case gives an example of rapid evolution of renal failure in the lack of treatment. 62 years old man, Armenian, consults  a physician with such complaints as evening fever up to 38° for a month, frequent urination and dark urine. Biochemical blood test revealed an increase in the following indices: creatinine-489 μmol / l, urea 28.3 μmol / l, uric acid 619 μmol / l. GFR-6ml / min. No amyloid deposits were detected by biopsy of the buccal mucosa. The diagnosis of FMF, chronic kidney disease (CKD) stage 5. Initiation of treatment. Tenkhoff catheter installation. Treatment includes сontinuous ambulatory peritoneal dialysis (CAPD) in mode 4 exchanges with a glucose solution of 1.36% 2.0 liters. The dynamics of treatment is positive in  presence of constant replacement therapy. A feature of this clinical case is the insidious development of secondary (AA) amyloidosis in uncontrolled patient, which leads to the development of end-stage chronic kidney disease (CKD). This case demonstrates the need of constant examination and treatment of the patients with end-stage CKD by renal replacement therapy, which significantly reduces the quality of patient`s life.

References

Ben-Zvi I, Herskovizh C, Kukuy O, Kassel Y, Grossman C, Livneh A. Familial Mediterranean fever without MEFV mutations: a case-control study. Orphanet J Rare Dis. 2015 Mar 25;10:34.. PMID: 25887307; PMCID: PMC4377009.

Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study.Medicine (Baltimore). 2005 Jan;84(1):1-11. PMID: 15643295.

Gattorno M, Hofer M, Federici S, & Ruperto N; Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO). Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis. 2019 Aug;78(8):1025-1032. Epub 2019 Apr 24. PMID: 31018962.

Graňák K, Jeseňák M, Vnučák M, Skálová P, Laca Ľ, Mokáň M, Dedinská I. Kidney transplantation as a treatment of choice for AA amyloidosis due to periodic fever syndrome. Clin Rheumatol. 2021 Feb;40(2):763-768. Epub 2020 Jul 11. PMID: 32654081.

Kucuk A, Gezer IA, Ucar R, Karahan AY. Familial Mediterranean Fever. Acta Medica (Hradec Kralove). 2014;57(3):97-104. PMID: 25649364.

Magaki S, Parks R, Vinters HV, Khanlou N. A 44-Year-Old Female with Familial Mediterranean Fever, Cardiomyopathy and End Stage Renal Disease. Brain Pathol. 2018 Jan;28(1):135-136. PMID: 29265632; PMCID: PMC6130257.

Siligato R, Gembillo G, Calabrese V, Conti G, Santoro D. Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever. Medicina (Kaunas). 2021 Oct 1;57(10):1049. PMID: 34684086; PMCID: PMC8541210.

Published
2021-12-01
How to Cite
1.
Palchukovska M, Liksunova L. FAMILIAL MEDITERRANEAN FEVER COMPLICATED BY CHRONIC KIDNEY DISEASE STAGE 5 DESCRIPTION OF THE CLINICAL CASE. USMYJ [Internet]. 2021Dec.1 [cited 2026Mar.21];4(127):44-7. Available from: https://mmj.nmuofficial.com/index.php/journal/article/view/343