Long-term outcome of renal transplantation in patients with familial Mediterranean fever amyloidosis: A single-center experience

Keven, Kenan; Şengül, Şule; Kutlay, Sim; Ekmekci, Yakup; Anadol, E.; Nergızoğlu, Gökhan; Ateş, Kenan; Ertürk, Şehsuvar; Erbay, Bülent

doi:10.1016/j.transproceed.2004.09.065

Cited by 32 publications

(12 citation statements)

References 5 publications

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“…The patient and allograft survivals vary substantially in these series, with outcomes that were worse (95) as well as outcomes that were similar (96,97) compared with the general renal transplant population. Recurrence of AA amyloidosis in the allograft occurred in 71% in one series (98).…”

Section: Amyloidosis-associated Esrdmentioning

confidence: 99%

Amyloidosis-Associated Kidney Disease

Dember

2006

Journal of the American Society of Nephrology

349

284

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The amyloidoses are a group of disorders in which soluble proteins aggregate and deposit extracellularly in tissues as insoluble fibrils, causing progressive organ dysfunction. The kidney is one of the most frequent sites of amyloid deposition in AL, AA, and several of the hereditary amyloidoses. Amyloid fibril formation begins with the misfolding of an amyloidogenic precursor protein. The misfolded variants self-aggregate in a highly ordered manner, generating protofilaments that interact to form fibrils. The fibrils have a characteristic appearance by electron microscopy and generate birefringence under polarized light when stained with Congo red dye. Advances in elucidating the mechanisms of amyloid fibril formation, tissue deposition, and tissue injury have led to new and more aggressive treatment approaches for these disorders. This article reviews the pathogenesis, diagnosis, clinical manifestations, and treatment of the amyloidoses, focusing heavily on the renal aspects of each of these areas.

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Section: Amyloidosis-associated Esrdmentioning

confidence: 99%

Amyloidosis-Associated Kidney Disease

Dember

2006

Journal of the American Society of Nephrology

349

284

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“…10 In many studies, graft and patient survival rates at 1, 5, and 10 years after transplant were similar in patients with FMF and other endstage kidney disease causes, and graft outcome rates at 1 and 10 years have been reported as over 90% and 70% in all series without exception. 4,[11][12][13]15 In our series, within the average 7-year follow-up, graft survival (censored for death) was over 90%.…”

Section: Discussionmentioning

confidence: 54%

“…In these patients, posttransplant lethal infections and recurrence of amyloidosis with cardiac involvement are serious complications. 4,5 The pathogenesis of infections in renal transplant patients with FMF is not known exactly. However, it is postulated that mutations in the Mediterranean fever MEFV gene, by simultaneously reducing the ability of pyrin to control interleukin 1β activation, may lead to inflammation.…”

Section: Introductionmentioning

confidence: 99%

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Karataş²,

Aykaş³

et al. 2017

Exp Clin Transplant

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Objectives: We evaluated long-term results and infections requiring hospitalization in kidney transplant patients with Familial Mediterranean Fever (associated amyloidosis-type). Materials and Methods:We retrospectively reviewed medical records of patients with familial Mediterranean fever with at least 1-year posttransplant follow-up. Kidney transplant recipients with primary glomerulonephritis and equivalent demography, immunity status, and follow-up comprised the control group. Results: In 32 patients with familial Mediterranean fever versus 25 control patients (mean follow-up 82 ± 57 vs 79 ± 54 mo; P = .82), average serum creatinine values were 1.7 ± 0.9 versus 1.5 ± 1.0 mg/dL (P = .41) at discharge, 1.4 ± 0.4 versus 1.3 ± 0.5 mg/dL (P = .44) at 1 year, 1.4 ± 0.6 versus 1.3 ± 0.5 mg/dL (P = .63) at 3 years, and 2.0 ± 1.5 versus 2.1 ± 1.5 mg/dL (P = .92) at last follow-up. Groups were not statistically different regarding average inpatient and number of hospitalizations due to infections at 1 year; however, at last follow-up, 26 patients with familial Mediterranean fever (81%) had 8.6 average admissions and 13 control patients (52%) had 2.8 average admissions (P = .02, P < .01). Early posttransplant, both groups were taking a triple drug immunosuppression regimen. However, at 1 and 3 years posttransplant, withdrawal and/or minimization occurred in 40.6% and 83.3% of patients with familial Mediterranean fever and 28% and 55.5% of control patients (P < .05, P < .05). During follow-up, 6 familial Mediterranean fever patients (18.7%) and 2 control patients (8%) died (P = .23). Conclusions: Although renal transplant patients with associated amyloidosis-type familial Mediterranean fever and those with glomerulonephritis have similar rejection and/or graft loss rates, hospital admissions due to infection and increased mortality are more common in the familial Mediterranean fever group, with immunosup pression drug withdrawal or switching due to infection more often required.

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“…7 Kidney transplant is often uneventful in these patients and does not differ in terms of patient outcomes from recipients with primary renal disease of a different cause. [7][8][9][10] There are insufficient data in the literature about primary disease recurrence in FMF patients after renal transplant. In 2 small-scale studies from Turkey 8 and Iran 9 that evaluated the long-term consequences of FMF after kidney transplant, the investigators detected relapses in 2 of 17 patients 8 and 1 of 18 patients.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5][6] In clinical practice, patients with FMF often present with AA amyloidosis-mediated endstage renal disease (ESRD) but generally recover after kidney transplant. [7][8][9][10] Fever is a common feature in patients who undergo transplant and is commonly associated with infectious diseases, but less often with lymphoproliferative disorders, cancer or graft rejection. [11][12][13] However, fever of unknown origin (FUO) is a rare condition after solid-organ transplant and is generally associated with atypical infections (eg, tuberculosis, fungal infections) and/or lymphoproliferative disorders.…”

Section: Introductionmentioning

confidence: 99%

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Uslu²,

Şimşek³

et al. 2017

Exp Clin Transplant

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Fever of unknown origin is a rare condition after solidorgan transplant and is generally associated with atypical infections (eg, tuberculosis, fungal infections) and/or lymphoproliferative disorders. Here, we present a kidney transplant patient with a late diagnosis of E148Q mutation-positive familial Mediterranean fever as the cause of fever of unknown origin. A 22-year-old female patient with a previous history of 4 years of hemodialysis and unknown primary renal disease received a deceased-donor kidney transplant at our center 5 years previously. She had an uneventful course in the first 3 years following transplant. After this period, she was hospitalized 3 times during a 4-month period with fever, nausea, vomiting, and atypical abdominal pain. At that time, hemogram results were unremarkable, except for mild leukocytosis and slightly elevated acute-phase reactants; blood, urine, and throat cultures were negative, and there were no remarkable findings on imaging tests. Fever was controlled within 48 hours by administering empiric ampicillin-sulbactam therapy and discon tinuing immunosuppressive treatment except steroids. Three successive hospital admissions owing to similar complaints suggested periodic fever syndrome, and therapy with 1 g/day colchicine led to an excellent clinical response with no recurrence of fever or other symptoms. An FMF gene mutation analysis revealed heterozygous E148Q mutation positivity. Continuing the current treatment regimen, the patient did well during at approximately 1.5 years of follow-up. In the Mediterranean region population, familial Mediterranean fever should be considered in the diagnosis of fever of unknown origin in patients who have undergone renal transplant. E148Q mutation-positive familial Mediterranean fever has a subclinical course and renal manifestations that differ from AA amyloidosis during childhood and may be responsible for de novo familial Mediterranean fever after renal transplantation.

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Long-term outcome of renal transplantation in patients with familial Mediterranean fever amyloidosis: A single-center experience

Cited by 32 publications

References 5 publications

Amyloidosis-Associated Kidney Disease

Amyloidosis-Associated Kidney Disease

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