Malignant Nephrosclerosis in a Patient with Familial Mediterranean Fever

Masayuki Yamanouchi; Yoshifumi Ubara; Aya Imafuku; Masahiro Kawada; Mise Koki; Keiichi Sumida; Rikako Hiramatsu; Eiko Hasegawa; Noriko Hayami; Tatsuya Suwabe; Junichi Hoshino; Naoki Sawa; Kenichi Ohashi; Takeshi Fujii; Masayuki Matsuda; Kenmei Takaichi

doi:10.2169/internalmedicine.54.4937

CASE REPORTS

Malignant Nephrosclerosis in a Patient with Familial Mediterranean Fever

Masayuki Yamanouchi, Yoshifumi Ubara, Aya Imafuku, Masahiro Kawada, Mise Koki, Keiichi Sumida, Rikako Hiramatsu, Eiko Hasegawa, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenichi Ohashi, Takeshi Fujii, Masayuki Matsuda, Kenmei Takaichi

Author information

Masayuki Yamanouchi
Nephrology Center, Toranomon Hospital, Japan
Yoshifumi Ubara
Nephrology Center, Toranomon Hospital, Japan
Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan
Aya Imafuku
Nephrology Center, Toranomon Hospital, Japan
Masahiro Kawada
Nephrology Center, Toranomon Hospital, Japan
Mise Koki
Nephrology Center, Toranomon Hospital, Japan
Keiichi Sumida
Nephrology Center, Toranomon Hospital, Japan
Rikako Hiramatsu
Nephrology Center, Toranomon Hospital, Japan
Eiko Hasegawa
Nephrology Center, Toranomon Hospital, Japan
Noriko Hayami
Nephrology Center, Toranomon Hospital, Japan
Tatsuya Suwabe
Nephrology Center, Toranomon Hospital, Japan
Junichi Hoshino
Nephrology Center, Toranomon Hospital, Japan
Naoki Sawa
Nephrology Center, Toranomon Hospital, Japan
Kenichi Ohashi
Department of Pathology, Toranomon Hospital, Japan
Takeshi Fujii
Department of Pathology, Toranomon Hospital, Japan
Masayuki Matsuda
Department of Internal Medicine (Neurology and Rheumatology), Shinshu University, Japan
Kenmei Takaichi
Nephrology Center, Toranomon Hospital, Japan
Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan

Keywords: MEFV gene, E148Q mutation, malignant nephrosclerosis, familial Mediterranean fever, hemodialysis

JOURNAL OPEN ACCESS

2015 Volume 54 Issue 20 Pages 2643-2646

DOI https://doi.org/10.2169/internalmedicine.54.4937

Details

Abstract

A 37-year-old man was admitted to our hospital for an evaluation of renal dysfunction and hypertension. The C-reactive protein level was 6.0 mg/dL, and the serum renin activity was extremely high. A renal biopsy showed malignant nephrosclerosis-like lesions with an onion skin pattern. He had a history of recurrent abdominal pain associated with periodic fevers above 38 degrees that resolved within three days. A MEditerranean FeVer (MEFV) gene analysis revealed that he was homozygous for the E148Q polymorphism (exon 2) and heterozygous for the L110P polymorphism (exon 2). The present case demonstrates that persistent subclinical inflammation can lead to malignant nephrosclerosis in familial Mediterranean fever patients with this genotype.

Corresponding author

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