Inflammasome and immune disorders (97)
Autoimmune and Inflammatory Disorders Research (38)
interferon and immune responses (32)
Immunodeficiency and Autoimmune Disorders (28)
Immune Cell Function and Interaction (27)
Oshima K, Inage E, Toriumi S, Oishi K, Yamada H , et al.
The Tohoku journal of experimental medicine •
Cimetidine is effective for treating periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, primarily in children, and is covered by public insurance in Japan. However, as demand for gastric ulcer treatment declines, cimetidine production has been restricted, raising concerns about supply for pediatric patients. We analyzed Japanese prescription trends in adults and children using publicly available data. Specifically, national reimbursement data were used to assess annual cimetidine prescription volumes by age group and pediatric share. Chronological changes were examined based on original formulation supply. In the database, 11 and 6 cimetidine products (including discontinued stock) were available for outpatient and in-hospital dispensing, respectively. Annually, 1.01 million and 80,000 cimetidine tablets (200-mg equivalents) were dispensed for patients under 15 years in outpatient and in-hospital settings, respectively. No inpatient prescriptions were recorded. Pediatric prescriptions peaked at ages 5-9 years, differing from adult trends, and accounted for 2.5% of total prescriptions. Although overall dispensing volumes are declining, pediatric prescriptions are increasing. Despite overall demand decreasing, approximately 1 million 200-mg cimetidine tablets are needed annually for use in children, and pediatric demand is increasing. Therefore, securing production of this new indication is essential.
Kutsuna S, Ohmagari N, Tanizaki R, Hagino N, Nishikomori R , et al.
Modern rheumatology •
A 26-year-old woman presented with fever and pharyngitis. She previously experienced four periodic febrile episodes at 30- to 40-day intervals. We suspected periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, and prescribed predisolone, thereby her fever rapidly subsided. Her febrile episodes improved after daily cimetidine treatment. Genetic testing results of genomic DNA for periodic fever syndromes were negative, although she was heterozygous for p.Glu148Gln variation in MEFV, supporting the diagnosis of PFAPA syndrome.
Taniuchi S, Nishikomori R, Iharada A, Tuji S, Heike T , et al.
The open rheumatology journal •
The pathogenesis of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome is unknown as yet. In order to understand whether genes implicated in other auto-inflammatory diseases might be involved in the pathogenesis of PFAPA, all variants in the genes causing familial Mediterranean fever (FMF), tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS), and Hyper IgD syndrome were analyzed in children with PFAPA. All variants in MEFV, TNFRSF1A, and MVK were analyzed in 20 patients with PFAPA. PFAPA were diagnosed by previous published criteria. The findings of all analyses in PFAPA patients were compared with those of unaffected normal subjects (n=62). In the 13 children of 20 with PFAPA, the heterozygous variants of MEFV (5 patients: E148Q-L110P, 2 patients: E148Q, 1 patient: E148Q-L110P/E148Q, 1 patient: E148Q-P369S-R408Q-E84K, 1 patient: E148Q-L110P-P369S-A408G, 1 patient: R202Q, 1 patient: P115R) were found. No variants belonging to TNFRSF1A or MVK were detected in children with PFAPA. The frequency of the E148Q-L110P variants in children with PFAPA was significantly higher than that observed in unaffected normal subjects (7/20 versus 8/62). The duration of the episodes of illness in PFAPA children with MEFV variants was shorter than that of patients without variants. Genes involved in the development and progression of MEFV may affect the incidence and the phenotype of PFAPA in children.