PFAPA (Marshall Syndrome). Diagnosis Improvement after Implementation of Awareness Programm

Background. PFAPA is a chronic auto-inflammatory disease including recurrent fever, aphthae, pharyngitis, adenitis. Steroids use from beginning of episode is able to stop it and no antibiotherapy is recommended. Objectives. Authors analyzed the results after dissemination of information about PFAPA patients among general practitioners (GPs) in our county, in order not only to improve diagnosis but also to avoid unnecessary antibiotherapy. Methods. Authors analyzed PFAPA children before and after implementation of a plan concerning improving PFAPA knowledge. Authors compared 2 PFAPA-groups: first group (patients diagnosed before programm implementation) and the second group (patients enrolled after programm application). Both groups were enrolled during 12 month-period and were evaluated according to same criteria: symptoms onset age, duration between episodes and period between disease onset and its confirmation. The programme was implemented in 2015 and comprised 4 meetings together with GPs. The patients were included in first group between january–december 2014. From january to december 2016 were enrolled patients belonging to second group. Patients up to 10 year-age fulfilling diagnosis criteria were analyzed. Results. 79 patients were included in study: 18 patients (first group) and 61 PFAPA-children (second group). Mean age disease debut was 42.23 months (1^st group) and 28.13 months for the 2^nd. Duration from symptoms onset to diagnosis confirmation was 36.29 months (1^st group) and 11.29 months for the second group, as a consequence of initiation of PFAPA awareness programm. Average period between fever attacks was 7.1 weeks (1^st group) and 7.3 (2^nd group) respectively. Conclusions. 1.Authors remarked a low suspicion index for PFAPA diagnosis among GPs before plan initiation; 2.PFAPA diagnosis war significantly improved after programm implementation (61 versus 18 cases); 3.PFAPA awareness programm was very useful and has to continue in order to avoid unjustified antibiotics, in the future, for PFAPA patients.