Immunoglobulin A nephropathy associated with familial mediterranean fever

Abstract

Familial Mediterranean fever (FMF) is a rare autosomal recessive autoinflammatory disorder, characterized by recurrent episodes of transient fever and polyserositis. Nonamyloid renal involvement has been reported rarely in patients with FMF, such as immunoglobulin A (IgA)nephropathy, in childhood. We report a 7-year-old girl, who was first seen at 1.5 years of age with nephrotic range proteinuria and edema. Based on renal histological demonstration of mesangial proliferation and mesangial IgA deposits, a diagnosis of IgA nephropathy was made. Following recurrent episodes of fever, abdominal pain, and bone pain, a diagnosis of periodic fever was suspected. A known homozygous mutation (p. P369S) was detected in exon 3 of MEPV, the gene encoding pyrin, indicating the diagnosis of FMF. Hence, FMF and IgA nephropathy might coexist. Kidney biopsy is indicated in patients with FMF and early onset of proteinuria to rule out renal involvement with amyloidosis or glomerulonephritis to enable decisions on management.