We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Late-onset familial Mediterranean fever in Japan.
- Authors
Dai Kishida; Masahide Yazaki; Akinori Nakamura; Ayako Tsuchiya-Suzuki; Yasuhiro Shimojima; Yoshiki Sekijima
- Abstract
Objectives: Most patients with familial Mediterranean fever (FMF) have their first attack at age < 20 years. Information about late-onset (age ≥40 years) FMF is limited. We aimed to evaluate the demographic, clinical, and genetic characteristics of late-onset FMF patients in the Japanese population. Methods: We retrospectively analyzed 292 patients with FMF. Patients were divided into three groups according to age of disease onset: Group I, ≤19 years; Group II, 20-39 years; and Group III, ≥40 years. Results: Of 292 patients, 44 (15.1%) experienced their first attack at age ≥40 years. While high fever (97.7%) and arthritis (45.5%) were common symptoms in Group III patients, peritonitis (40.9%) and pleuritis (25.0%) were significantly lower than in other groups. The frequency of patients carrying p.M694I (18.2%), which is the most representative mutation in Japan, was significantly lower in Group III than in Group I. The response to colchicine therapy was good (95.1%) and similar in all groups. Conclusions: In Japan, more patients than expected had late-onset FMF. They had a milder form of disease, with less frequent peritonitis and pleuritis. The response to colchicine treatment was good. Clinicians should consider FMF for patients with unexplained recurrent febrile episodes, regardless of age.
- Subjects
FAMILIAL Mediterranean fever; COLCHICINE; FEVER; GENETIC mutation; PERITONITIS; PLEURISY
- Publication
Modern Rheumatology, 2020, Vol 30, Issue 3, p564
- ISSN
1439-7595
- Publication type
Article
- DOI
10.1080/14397595.2019.1621440