title: Malignant nephrosclerosis in a patient with familial Mediterranean fever.
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.
authors.affiliation: Nephrology Center, Toranomon Hospital, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yamanouchi
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
authors.forename: Aya
authors.initials: A
authors.lastname: Imafuku
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kawada
authors.forename: Mise
authors.initials: M
authors.lastname: Koki
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.forename: Rikako
authors.initials: R
authors.lastname: Hiramatsu
authors.forename: Eiko
authors.initials: E
authors.lastname: Hasegawa
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fujii
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
mesh_terms: D015746:Abdominal Pain / Q000150:complications; D000328:Adult; D002097:C-Reactive Protein / Q000032:analysis; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000235:genetics*; D005334:Fever / Q000150:complications; D005838:Genotype; D006801:Humans; D007249:Inflammation / Q000150:complications; D008297:Male; D009400:Nephrosclerosis / Q000150:complications / Q000209:etiology*; D011110:Polymorphism, Genetic; D012083:Renin / Q000097:blood
chemical_list: D002097:C-Reactive Protein; D012083:Renin