Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults

Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a clockwork periodicity. Although rare after adolescence, adult patients with PFAPA syndrome may present with a wider range of symptoms and may lack the clockwork periodicity of fever. A 24-year-old patient presented with a 4-year history of periodic fever with pharyngitis and cervical adenitis. She also complained of vomiting, fatigue and sporadically presented with aphthous stomatitis. During crises, laboratory evaluation showed a moderate elevation of inflammatory markers. Blood cultures and ANA titres were negative. Immunoglobulins and serum ferritin levels were normal. After other causes of periodic fever had been excluded, a diagnosis of PFAPA syndrome was made. LEARNING POINTS: PFAPA syndrome is characterized by periodic fever accompanied by pharyngitis, aphthous stomatitis and cervical adenitis. It is a common cause of periodic fever in children but can also present in adults with a wider range of clinical manifestations. Establishing a firm diagnosis of PFAPA syndrome may avoid excessive work-up and potentially harmful treatment.