Long-Term Outcome of Renal Transplantation in Patients With Familial Mediterranean Fever Amyloidosis: A Single-Center Experience

• Published in: Transplantation proceedings 2013-12, Vol.45 (10), p.3502-3504
• Main Authors: Abedi, A.S, Nakhjavani, J.M, Etemadi, J
• Format: Article
• Language: English
• Subjects: Adult; Amyloidosis - diagnosis; Amyloidosis - etiology; Amyloidosis - mortality; Amyloidosis - prevention & control; Colchicine - adverse effects; Cytoskeletal Proteins - genetics; DNA Mutational Analysis; Familial Mediterranean Fever - complications; Familial Mediterranean Fever - diagnosis; Familial Mediterranean Fever - drug therapy; Familial Mediterranean Fever - genetics; Familial Mediterranean Fever - mortality; Female; Genetic Predisposition to Disease; Graft Survival; Heterozygote; Homozygote; Humans; Iran; Kidney Failure, Chronic - diagnosis; Kidney Failure, Chronic - etiology; Kidney Failure, Chronic - mortality; Kidney Failure, Chronic - surgery; Kidney Transplantation - adverse effects; Kidney Transplantation - mortality; Male; Middle Aged; Mutation; Phenotype; Pyrin; Risk Factors; Secondary Prevention; Surgery; Survival Analysis; Time Factors; Treatment Outcome
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2013.09.021

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.