D1.326 - Six Cases of Adult-Onset PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, and Cervical Adenopathy) Syndrome
Case report
The frequency of adult-onset PFAPA syndrome cases is increasing. Diagnosis is usually made with clinical findings and laboratory tests. In all the cases listed, acute phase reactants were high during the attacks, while they were normal in the attack-free period.
Case 1: A 31-year-old male patient presented with recurrent fever, oral aphthae, and white plaques in the throat for 3 years, occurring once a month. There was no response to antibiotic therapy, and arthralgia was present before the onset of lesions. Short-term methylprednisolone and colchicine treatment was started. The frequency and duration of attacks decreased with colchicine.
Case 2: A 33-year-old male patient with a history of tonsillectomy was examined for recurrent stomatitis and cervical lymphadenopathy for 5 years. Attacks were controlled with colchicine.
Case 3: A 20-year-old male patient presented with complaints of sore throat, high fever, and mouth sores recurring every 14 days. He had a history of antibiotic use due to frequent upper respiratory tract infections. High levels of IgG1 and IgG4 were detected. Response to steroids was proven.
Case 4: An 18-year-old male patient presented to our outpatient clinic with mouth sores and high fever recurring every 14 days for 2 years. He had a history of asthma and venom allergy. Colchicine was started with a diagnosis of PFAPA, and there were no attacks in his follow-ups.
Case 5: A 25-year-old female patient presented with high fever accompanied by recurrent headache, fatigue, sore throat, difficulty swallowing, and mouth sores every 4-5 weeks. She was getting partial benefit from antibacterial treatments. She also had frequent upper respiratory tract infections without aphthae and sore throat. Selective IgA deficiency was also diagnosed in immunological evaluation. Antistreptolysin O was negative. Her complaints regressed with corticosteroids. Regular colchicine treatment was planned.
Case 6: A 21-year-old female patient presented with fever, malaise, fatigue, sore throat, cervical lymphadenopathies, oral aphthae, and erythema nodosum-like skin rash and genital aphthae accompanying some attacks, occurring every 6-8 weeks. Pathergy was negative, there was no uveitis, and HLA B51 was negative. Bacteriological examination was negative. Ig levels were normal. Tonsillectomy was recommended to the patient who had a good response to steroids and did not accept regular colchicine treatment.
