Renal transplantation in amyloidosis secondary to familial Mediterranean fever

Abstract

FAMILIAL Mediterranean fever (FMF) is an autosomal recessive disorder, common in some ethnic groups such as Sephardic Jews, Armenians, Turks, and Arabs. The disease is characterized by recurrent attacks of fever associated with serositis affecting the pleura, peritoneum, and/or large joints as well as systemic amyloidosis of the AA type causing nephropathy and end-stage renal failure (ESRF). There is a wide variation in the reporting of this disease as a cause of ESRF. Among the member countries of European Dialysis and Transplantation Association (EDTA), amyloidosis was responsible 1.6% of all ESRF patients. In 76% of these patients, chronic inflammatory diseases were the underlying etiology for amyloidosis, and FMF was the second cause. Most of the FMF patients were localized to Egypt, Israel, and Turkey. Even a lower incidence (0.3%) of amyloidosis as a cause of ESRF has been reported from the United States; however the underlying etiology of these cases was not defined in this report. In contradistinction, amyloidosis represents a more common cause of chronic renal failure among patients from the Middle East and the Mediterranean countries; and FMF has been identified as the most frequent underlying cause for the disease in these groups. Indeed, FMF amyloidotic patients represent 6% of the candidates for renal transplantation in Israel. In Turkey 4.2% of ESRF patients suffer from amyloidosis, although the underlying etiology was not identified in this report. Thus, at least in some countries, amyloidosis is an important etiologic factor for ESRF. As most of the patients with renal amyloidosis progress to ESRF, the selection of the renal replacement therapy modality (ie, hemodialysis, peritoneal dialysis, or renal transplantation) is a major concern. It has been reported that amyloidotic patients maintained on chronic hemodialysis experience higher rates of morbidity and mortality when compared to dialysis patients whose causes of ESRF were due to other diseases. There is not enough information regarding the patients on chronic peritoneal dialysis; because the effects of repeated peritoneal inflammation on peritoneal permeability has not been well defined. Considering kidney transplantation, many authors have concerns, because amyloidosis is a generalized disorder and most patients show systemic manifestations of the disease. There are four concerns when considering these patients for transplantation.