Abstract
PFAPA Syndrome (Periodic Fever, Aphthous stomatitis, Pharingitis, and cervical Adenitis) is the most common periodic fever in childhood; the diagnosis is based on clinical criteria. Familiar Mediterranean Fever (FMF) is a monogenic autosomal recessive autoinflammatory disease, whose diagnosis is based on clinical elements, supported by MEFV genetic mutations. When there is only a mutation or no one, the patient undergoes a trial with colchicine for 4-6 months, and diagnosis is confirmed in case of clinical response and fever early recurrence after suspension. Current treatment of PFAPA is symptomatic. Febrile episodes show a rapid response to the administration of one or two doses of prednisone (1-2 mg/kg) or betamethasone (0.1-0.2 mg/kg). Total requirement of steroid increases over time, and the frequency of attacks worsens the quality of life of patients. In literature, the prophylaxis of PFAPA febrile attacks with colchicine (0.5-1 mg/day) has been tested only on a few patients, with controversial results.
Highlights
PFAPA Syndrome (Periodic Fever, Aphthous stomatitis, Pharingitis, and cervical Adenitis) is the most common periodic fever in childhood; the diagnosis is based on clinical criteria
Familiar Mediterranean Fever (FMF) is a monogenic autosomal recessive autoinflammatory disease, whose diagnosis is based on clinical elements, supported by MEFV genetic mutations
When there is only a mutation or no one, the patient undergoes a trial with colchicine for 4-6 months, and diagnosis is confirmed in case of clinical response and fever early recurrence after suspension
Summary
PFAPA Syndrome (Periodic Fever, Aphthous stomatitis, Pharingitis, and cervical Adenitis) is the most common periodic fever in childhood; the diagnosis is based on clinical criteria. Familiar Mediterranean Fever (FMF) is a monogenic autosomal recessive autoinflammatory disease, whose diagnosis is based on clinical elements, supported by MEFV genetic mutations. When there is only a mutation or no one, the patient undergoes a trial with colchicine for 4-6 months, and diagnosis is confirmed in case of clinical response and fever early recurrence after suspension. Febrile episodes show a rapid response to the administration of one or two doses of prednisone (1-2 mg/kg) or betamethasone (0.1-0.2 mg/kg). The prophylaxis of PFAPA febrile attacks with colchicine (0.5-1 mg/day) has been tested only on a few patients, with controversial results
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call