A Case of Familial Mediterranean Fever without Fever

: Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease commonly observed around the Mediterranean basin presenting as recurrent febrile episodes. We herein describe a Japanese case of genetically-confirmed FMF, in which fever was lacking during attacks. An otherwise healthy 34-year-old man presented with frequent episodes of abdominal pain, which resolved spontaneously. During the attacks, the patient was afebrile, but the inflammatory marker levels in his blood were increased. Abdominal CT dem-onstrated enhancement of the jejunal membrane. After the initiation of colchicine therapy, the patient experi-enced no attacks for more than one year. The diagnosis of FMF was confirmed by a genetic analysis.

[1]  Marwa Said,et al.  Molecular Patterns of MEFV Gene Mutations in Egyptian Patients with Familial Mediterranean Fever: A Retrospective Cohort Study , 2019, International journal of inflammation.

[2]  D. Kastner,et al.  Pyrin Inflammasome Activation and RhoA Signaling in the Autoinflammatory Diseases FMF and HIDS , 2016, Nature Immunology.

[3]  N. Wulffraat,et al.  Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever , 2015, Annals of the rheumatic diseases.

[4]  A. Gül Treatment of familial Mediterranean fever: colchicine and beyond. , 2014, The Israel Medical Association journal : IMAJ.

[5]  Y. Berkun,et al.  Diagnostic criteria of familial Mediterranean fever. , 2014, Autoimmunity reviews.

[6]  A. Nakamura,et al.  Clinical and Genetic Features of Familial Mediterranean Fever in Japan , 2009, The Journal of Rheumatology.

[7]  B. Eser,et al.  Analysis of familial Mediterranean fever gene mutations in 202 patients with familial Mediterranean fever. , 2008, Genetic testing.

[8]  A. Bakkaloğlu,et al.  Familial Mediterranean Fever (FMF) in Turkey: Results of a Nationwide Multicenter Study , 2005, Medicine.

[9]  Isabelle Touitou,et al.  The spectrum of Familial Mediterranean Fever (FMF) mutations , 2001, European Journal of Human Genetics.

[10]  D. Zemer,et al.  Criteria for the diagnosis of familial Mediterranean fever. , 1997, Arthritis and rheumatism.

[11]  E. Sohar,et al.  Familial Mediterranean fever. , 1955, Klinische Padiatrie.

[12]  D. Zemer,et al.  Colchicine in the prevention and treatment of the amyloidosis of familial Mediterranean fever. , 1986, The New England journal of medicine.

[13]  E. Sohar,et al.  FAMILIAL MEDITERRANEAN FEVER , 1959, Definitions.

[14]  S. Siegal Benign paroxysmal peritonitis. , 1945, Gastroenterology.

[15]  T. C. Janeway,et al.  AN UNUSUAL PAROXYSMAL SYNDROME, PROBABLY ALLIED TO RECURRENT VOMITING,: WITH A STUDY OF THE NITROGEN METABOLISM. , 1908 .