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Intern Med J. 2014 Nov;44(11):1128-33. doi: 10.1111/imj.12520.

Assessment of renal involvement in patients with familial Mediterranean fever: a clinical study from Ardabil, Iran.

Author information

1
Department of Internal Medicine, Imam Khomeini Hospital, Ardabil University of Medical Sciences, Ardabil, Iran.

Abstract

BACKGROUND AND AIM:

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent episodes of painful inflammation in the abdomen, chest or joints. The association between FMF and non-amyloid glomerulopathies are unusual. In this study, we describe our experiences and observations about renal involvement in patients with FMF.

METHODS:

A total of 108 patients with FMF was enrolled in the study. Twelve patients with FMF were referred to the Nephrology Service, for evaluation and assessment of the degree of renal involvement. All the 12 patients underwent percutaneous ultrasound-guided renal biopsies and genetic analysis.

RESULTS:

On microscopic examination of the kidney specimens, six patients were found to have amyloidosis, five focal segmental glomerulosclerosis and one patient membranoproliferative glomerulonephritis. It seems that in patients with FMF and renal amyloidosis, the response to treatment with colchicine is excellent, but in patients with FMF and focal segmental glomerulosclerosis, the response to treatment with colchicine is poor. We present an evidence-based algorithm, constructed based on literature review, to aid decision making in management of renal involvement in patients with FMF.

CONCLUSION:

The results of our study suggest that in patients with FMF and renal involvement, non-amyloid renal lesions should be considered in the differential diagnosis in addition to amyloidosis.

KEYWORDS:

amyloidosis; familial Mediterranean fever; renal involvement

PMID:
24989840
DOI:
10.1111/imj.12520
[Indexed for MEDLINE]

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