Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group

Abstract

Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA.

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review

“… 43 Amarilyo et al found that the majority of physicians use 1 mg/kg of prednisone to treat attacks, and recommend increasing to 2 mg/kg in the case of frequent fever flares or incomplete response to the lower dose. 15 The fever episode is usually aborted within hours, with a minority of patients requiring a second dose 8–12 h later. Other symptoms, such as aphthous ulcers, may subside more slowly.…”

“…Recently PFAPA CTPs were published by an international group of researchers from the CARRA (Childhood Arthritis and Rheumatology Research Alliance) PFAPA work group. 15 …”

“… 72 Finally, there is currently no evidence on the efficacy of tonsillotomy in patients with PFAPA. 15 …”

“… 12 , 13 , 14 Additionally, consensus treatment plans have been developed, which will aid in developing evidence-based therapeutic guidelines and establishing a standard of care. 15 Significant progress has been made particularly in the genetic realm, and a number of studies have been conducted to shed light on the potential hereditary factors involved in PFAPA. 16 , 17 , 18 , 19 …”

Colchicin: altes Medikament mit neuem Nutzen

“…Ein Konsens bezüglich Dosierung und Therapiedauer besteht nicht, da die Erkrankung in der Regel selbstlimitierend verläuft. Vorgeschlagen wird eine Schubprophylaxe mit 0,5–1,25 mg Colchicin pro Tag [ 50 ].…”