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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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Published on: February 8, 2019

[Clinical aspects of Familial Mediterranean fever]

Kiyoshi Migita1, Kazunaga Agematsu

  • 1Clinical Research Center, NHO Nagasaki Medical Center.

Nihon Rinsho Men'Eki Gakkai Kaishi = Japanese Journal of Clinical Immunology
|November 2, 2011
PubMed

Insights

Familial Mediterranean fever (FMF) is a rare autoinflammatory disease. In Japan, around 300 patients have FMF, with colchicine proving effective for most, highlighting the need for early diagnosis to prevent complications like AA amyloidosis.

Area of Science:

  • Rheumatology
  • Genetics
  • Internal Medicine

Background:

  • Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder.
  • Characterized by recurrent fever and serositis.
  • Prevalence in Japan is estimated at approximately 300 patients.

Purpose of the Study:

  • To report findings from a nationwide survey of FMF patients in Japan.
  • To describe the clinical characteristics and treatment outcomes.
  • To emphasize the importance of early diagnosis and treatment for preventing AA amyloidosis.

Main Methods:

  • Nationwide survey of Japanese FMF patients.
  • Data collection on symptoms, diagnosis, and treatment.
  • Analysis of patient demographics and clinical manifestations.

Main Results:

  • High-grade fever (95.5%), abdominal pain (62.7%), chest pain (35.8%), and arthritis (31.3%) were common symptoms.
  • AA amyloidosis was confirmed in 3.7% of patients.
  • Colchicine demonstrated high efficacy, with 91.8% of patients responding positively.

Conclusions:

  • A notable number of FMF patients reside in Japan.
  • Early diagnosis and prompt treatment are crucial for managing FMF.
  • Effective management, including colchicine therapy, can prevent severe complications such as AA amyloidosis.

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