Response to cimetidine in a 1-year-old child with PFAPA syndrome

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic febrile attacks recurring regularly every 2 to 8 weeks. Although tonsillectomy is offered as an effective treatment, the effectiveness of cimetidine treatment is still controversial. In this article, we describe two cases. A 1-year-old girl with PFAPA syndrome received cimetidine treatment and febrile attacks were reduced. Tonsillectomy was successfully performed on a 6-year-old boy with PFAPA syndrome. We suggest that the use of cimetidine may reduce febrile attacks of PFAPA syndrome until an age that is safer for tonsillectomy.

Colchicine versus cimetidine: the better choice for Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome prophylaxis, and the role of MEFV gene mutations

“…Thomas et al and Wurster et al recorded the effect of this drug on the prevention of febrile attacks in some PFAPA patients by studying their medical records [ 2 , 22 , 23 ]. Cimetidine has also been used in early infancy to control febrile attacks and postpone more invasive therapies, such as tonsillectomy [ 24 ]. According to our review of the past literature, it seems there is no clinical trial that evaluates the effect of cimetidine in the prevention of febrile attacks in the PFAPF syndrome.…”

Tonsillectomy as Treatment for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

“…Prednisolone is eff ective against the febrile attacks of PFAPA syndrome, although it shortens the interval between febrile attacks 7 . Cimetidine can reduce the febrile attacks of PFAPA syndrome 3,4,7,15 . Cimetidine is a common H2 antagonist that inhibits suppressor CD8+ T-lymphocyte activation and chemotaxis 6,7 .…”

“…We previously proposed the use of cimetidine to reduce the febrile attacks of PFAPA syndrome until the age of 3 years, at which time tonsillectomy can be performed 15 .…”

May cyproheptadine be a treatment choice for prophylaxis of PFAPA syndrome?

“…Cimetidine, a selective histamine-2 receptor antagonist was recommended to use for prophylaxis and reduce the attacks of PFAPA syndrome. It inhibits suppressor CD8+ T-lymphocyte activation and chemotaxis [5]. Histamine-2 receptor antagonist markedly decreased serotonin concentrations by the prolonged treatment.…”