Periodic fever syndrome with aphthous stomatitis, pharyngitis and cervical adenopathy treated with ketotifen – a case report

Toplak, Nataša; Glavnik, Vesna; Kuhar, Marijana; Avčin, Tadej

doi:10.1186/1546-0096-6-s1-p204

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“…According to some studies it reduces the production of IL-1b and some proinflammatory chemokines and modulates the T cell response. The positive effect of ketotifen on reduction of frequent attacks was also demonstrated in a case series of PFAPA patients (26). Another interesting approach is the application of probiotics containing Streptococcus salivarius K12, which produces bactericidal substances that can interact with the growth of many bacteria and modulate immune response in oral cavity.…”

Section: Introductionmentioning

confidence: 82%

“…We were inspired by case series published by Toplak et al. The rationale for such approach can be the fact that ketotifen reduces the production of IL-1β and some chemokines and modulates the T-cell response ( 26 ), which may have a positive effect on the immune dysregulation observed in PFAPA. Other studies investigating the effect of ketotifen also confirmed that ketotifen reduces the production or suppresses the secretion of the main pro-inflammatory cytokines, namely IL-1β, IL-6 and TNF-α ( 39 – 42 ).…”

Section: Discussionmentioning

confidence: 99%

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The use of ketotifen as long-term anti-inflammatory prophylaxis in children with PFAPA syndrome

Kapustova,

Banovcin,

Bobcakova

et al. 2023

Front. Immunol.

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IntroductionPeriodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA) is the most frequent periodic fever syndrome in children. Its pathogenesis is still unknown, but some disease-modifying factors were observed. Several medications were tested for the long-term prophylaxis of inflammatory flares; however, none are standardly used.MethodsThis prospective clinical trial enrolled 142 children (71 girls, 50%) meeting diagnostic criteria for PFAPA syndrome. We analysed selected clinical characteristics and compared laboratory parameters during the flare and attack-free period (at least two weeks after the attack). Moreover, we assessed the possible therapeutic effect of ketotifen on the duration of attack free-periods and clinical picture. ResultsThe mean age of patients was 6.81 ± 3.03 years and the mean age of onset of symptoms was 2.31 ± 2.02 years. No significant differences were observed between genders.We recorded a positive family history for PFAPA in 31.69% of patients. Attacks lasted for 2.8 ± 1.2 days, with intervals between attacks of 4 ± 1 weeks. We administered ketotifen in 111 (77.8%) patients, and a positive effect was observed in 86 (77.5%) of patients. We observed prolonged attack-free intervals in patients treated with ketotifen (14.7 ± 8.9 days in comparison with 4.4 ± 1.9 days before the treatment; p<0.001). The used dose of ketotifen was 0.08 ± 0.01 mg/kg/day. Mild side effects were observed in four patients (restlessness, irritability, agitation and constipation).DiscussionOur data supports the use of ketotifen for long-term prophylaxis in children with PFAPA syndrome with positive effects on the attenuation of disease activity and the prolongation of attack-free periods. Further well-designed studies should confirm the preliminary data.

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Section: Introductionmentioning

confidence: 82%