TY - JOUR T1 - Familial Mediterranean Fever (FMF) with Proteinuria: Clinical Features, Histology, Predictors, and Prognosis in a Cohort of 25 Patients JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.130520 SP - jrheum.130520 AU - Olga Kukuy AU - Avi Livneh AU - Aharon Ben-David AU - Juri Kopolovic AU - Alexander Volkov AU - Yael Shinar AU - Eliezer Holtzman AU - Dganit Dinour AU - Ilan Ben-Zvi Y1 - 2013/10/15 UR - http://www.jrheum.org/content/early/2013/10/09/jrheum.130520.abstract N2 - Objective Reactive (AA) amyloidosis may complicate familial Mediterranean fever (FMF), the prototype of autoinflammatory diseases. Thus, proteinuria in FMF is commonly viewed as resulting from amyloidosis, and kidney biopsy is deemed superfluous. However, nephropathy other than amyloidosis has been described in FMF, but its rate and distinctive characteristics are unknown. Our aim was to determine the rate and underlying pathology of FMF-related nonamyloidotic proteinuria and compare its clinical course, demographic, and genetic features to those of FMF-amyloid nephropathy. Methods This study is a retrospective analysis of data from patients with FMF undergoing kidney biopsy for proteinuria above 0.5 g/24 h, over 10 years (2001–2011). Clinical, laboratory, genetic, and pathology data were abstracted from patient files. Biopsies were viewed by an experienced pathologist, as necessary. Results Of the 25 patients referred for kidney biopsy, only 15 (60%) were diagnosed with amyloid kidney disease (AKD), and 10 were diagnosed with another nephropathy. The AKD and nonamyloid kidney disease (NAKD) groups were comparable on most variables, but showed distinct characteristics with regard to the degree of proteinuria (6.45 ± 4.3 g vs 2.14 ± 1.6 g, p = 0.006), rate of severe FMF (14 vs 5 patients, p = 0.022), and rate of development of end stage renal disease (73.3% vs 20%, p = 0.015), respectively. Conclusion NAKD is common in FMF and, compared to amyloidosis, it is featured with milder course and better prognosis. Contrary to common practice, it is highly recommended to obtain a kidney biopsy from patients with FMF and proteinuria more than 0.5 g/24 h. ER -