Elsevier

Transplantation Proceedings

Volume 45, Issue 10, December 2013, Pages 3502-3504
Transplantation Proceedings

13th Congress of the Middle East Society for Organ Transplantation (MESOT)
Renal transplantation
Long-Term Outcome of Renal Transplantation in Patients With Familial Mediterranean Fever Amyloidosis: A Single-Center Experience

https://doi.org/10.1016/j.transproceed.2013.09.021Get rights and content

Abstract

Introduction

Familial Mediterranean fever (FMF) is an autosomal-recessive disorder, affecting multiple organs. The AA type of amyloidosis is most common and serious complication cause nephropathy and end-stage renal disease (ESRD). Renal transplantation (RTX) remains treatment of choice for ESRD. We aimed to investigate long-term results of RTX in patients with FMF amyloidosis.

Patients and Methods

We compared the outcomes of 18 patients (12 men and 6 women) with FMF amyloidosis among 601 (2.9%) transplants with 200 control patients. Demographic data and gene analysis were evaluated.

Results

In our study the 1-year graft and patient survivals were 94.44% and 100%, respectively. At 5 years after RTX, they were 94.73% and 88.88%, respectively, in the FMF group without difference from controls. Mean creatinine level at 1 and 5 years were 1.43 ± 0.54 and 1.73 ± 0.89, respectively. The results of MEFV mutation analyses were: M694V/M694V homozygote in 1 patient, M694V/EQ148 in 3, M694V/V726A in 2, 680M-I/E148Q in 3, M694V/M680I in 5, R202Q/M680I in 2, and M694V/R202Q in 2. Recurrence was noticed in 1 patient with M694V/M680I. One patient died because of graft loss and cardiac complications with M694V/M680I gene analysis. Colchicine was reduced in 4 patients owing to side effects.

Conclusion

Long-term outcomes of transplantation in patients with amyloidosis secondary to FMF is similar to that in the general transplant population and maintenance colchicine, even after decreasing its dose, effectively prevents recurrence of amyloidosis in the allograft.

Section snippets

Patients And Method

We compared the results of RTX in 18 patients with FMF amyloidosis among 60 1(2.9%) transplants with 200 age- and gender-matched, non-FMF RTX patients as controls. In 2 groups, immunosuppression and duration of transplantation were similar. All patients has MEFV mutation analysis and renal biopsy before RTX. Blood urea, creatinine, urinalysis, and cyclosporine levels were measured monthly and 24-hour proteinuria every 3 months. Demographic data, transplantation duration, blood pressure,

Results

We included 18 patients (12 men and 6 women) with FMF amyloidosis with a mean age of 40.52 ± 7.1 years among 601 (2.9%) transplants with 200 controls (120 men and 80 women) with a mean age of 41.93 ± 10.93 years (P < .2). Mean transplantation duration was 76.42 ± 11.66 in FMF and 72 ± 12.14 in controls. Mean blood urea and creatinine in FMF group was 44.05 ± 9.34 and 1.43 ± 0.54 mg/dL 1 year after RTX without being different from controls (44.26 ± 10.4). Blood urea and 1.39 ± 0.30 creatinine (P

Discussion

FMF as an autosomal-recessive disease characterized with recurrent episodes of fever and serositis affects kidneys, by development of type AA amyloidosis causes proteinuria and nephropathy and leads to ESRD after 2 to 13 years.8

RTX remains treatment of choice for ESRD owing to FMF amyloidosis compared with hemodialysis or peritoneal dialysis, because of the higher morbidity and mortality rates of these patients on dialysis.2, 9 FMF accounted for 2% to 4% of all RTX in a study by Sherif et al2

References (13)

There are more references available in the full text version of this article.

Cited by (0)

View full text