Three Patients with Familial Mediterranean Fever: A Possible Underdiagnosed Entity in Japan

Yokota, Kazuhisa; Fukuda, Masakazu; Migita, Kiyoshi; Tanaka, Eiichiro; Okamoto, Tadashi; Kimura, Kenjiro

doi:10.2169/internalmedicine.53.2580

Intern. Med.

2014

DOI: 10.2169/internalmedicine.53.2580

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Three Patients with Familial Mediterranean Fever: A Possible Underdiagnosed Entity in Japan

Kazuhisa Yokota

Masakazu Fukuda

Kiyoshi Migita

et al.

Abstract: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by periodic fever and serosal inflammation. FMF is mostly reported in the Mediterranean region and is considered to be rare in Japan with estimated 292 cases. We treated three unrelated FMF patients in one year in a city with 144,000 residents. Two of the three patients were over 70 years old. FMF may therefore be underdiagnosed in Japan.

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“…test results were as follows: C-reactive protein (CRP): 89.9 mg/L (normal range: 0.00-5.00 mg/L), white blood cell count (WBC): 14,000/mm 3 (normal range: 4000-11,000/mm 3 ), hemoglobin: 11 mg/dL (normal range: 12-14 mg/dL), platelet count: 311,000/mm 3 (normal range: 150,000-400,000/mm 3 ). Biochemistry test results were within normal ranges.…”

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Coexistence of familial Mediterranean fever and Behçet’s disease: a case report

Güler¹

2017

Turk J Phys Med Rehab

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Behçet's disease (BD) is a chronic, multi-systemic vasculitis of every size and type vessels. It was first described by Hulusi Behçet, a Turkish Professor of Dermatology, in 1937, as a triad of recurrent aphthous stomatitis, genital aphthae, and relapsing uveitis. [1] The prevalence of BD is highest in the countries of the Eastern Mediterranean, Middle East, and Eastern Asian rim. [2] Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterized by periodic fever and serosal inflammation. [3] It is common in the Turkish, Armenian, Arabic, and Sephardic Jewish populations. [4] The Mediterranean Fever (MEFV) gene, associated with FMF, is located on chromosome 16 and encodes a 781-amino-acid protein termed pyrin. [5] Behçet's disease and FMF share a number of features in common in terms of clinical features and geographic distribution. Several studies have suggested that MEFV mutations may be associated with BD. [1,6-9] Herein, we report a case of coexistence of FMF and BD, and to discuss the immunopathogenetic mechanisms for possible associations among these two autoinflammatory diseases. CASE REPORT A 19-year-old female with a 10-year history of BD was referred to our outpatient rheumatology clinic with bilateral ankle joint arthritis, abdominal pain, and fever. The patient presented with a history of two years of recurrent attacks for fever, abdominal pain, and arthralgia for about four days. She was admitted to our emergency room with these complaints two days ago. She was followed for 24 hours, and no other pathology which could explain abdominal pain and infection findings for fever were able to be found. Therefore, she was referred to our outpatient rheumatology clinic. In the records of emergency room, fever was 39 °C, and laboratory ABSTRACT Behçet's disease (BD) is a chronic, multi-systemic vasculitis, characterized by a triad of recurrent aphthous stomatitis, genital aphthae, and uveitis. It is common in the Eastern Mediterranean, Middle East, and Eastern Asian countries. Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder, which is common seen in the Turkish, Armenian, Arabic, and Sephardic Jewish populations. It is characterized by recurrent episodes of fever, peritonitis, pleuritis, arthritis, and erysipelas-like skin lesions. Behçet's disease and FMF have common clinical features and geographic distribution. Herein, we present a 19-year-old female patient with coexistence of FMF and BD in the light of literature data.

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Coexistence of familial Mediterranean fever and Behçet’s disease: a case report

Güler¹

2017

Turk J Phys Med Rehab

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Three Patients with Familial Mediterranean Fever: A Possible Underdiagnosed Entity in Japan

Cited by 1 publication

References 12 publications

Coexistence of familial Mediterranean fever and Behçet’s disease: a case report

Coexistence of familial Mediterranean fever and Behçet’s disease: a case report

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