Periodic fever, aphthous stomatitis, pharyngitis and adenitis

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a medical syndrome characterized by recurrent episodes of fever, mouth sores, sore throat, and swollen lymph nodes. It is most commonly seen in children and is one of the most frequent causes of periodic fever in pediatric patients.

Symptoms

The primary symptoms of PFAPA include:

• Recurrent fever: Episodes typically last for 3-6 days and recur every 3-8 weeks.
• Aphthous stomatitis: Painful mouth ulcers that can affect the tongue, gums, and inside of the cheeks.
• Pharyngitis: Sore throat, often with redness and swelling.
• Adenitis: Swollen lymph nodes, particularly in the neck.

Diagnosis

PFAPA is diagnosed based on clinical criteria, as there are no specific laboratory tests for the syndrome. The criteria include:

• Regularly recurring fevers with an early age of onset (usually before 5 years old).
• Symptoms of aphthous stomatitis, pharyngitis, and adenitis.
• Exclusion of other causes of periodic fever, such as cyclic neutropenia, familial Mediterranean fever, and systemic juvenile idiopathic arthritis.

Treatment

Treatment options for PFAPA include:

• Corticosteroids: A single dose of prednisone or prednisolone can abort an episode.
• Tonsillectomy: Surgical removal of the tonsils may be considered in severe cases.
• Cimetidine: An H2 receptor antagonist that has been used with some success.

Prognosis

PFAPA is a benign condition, and most children outgrow the syndrome by adolescence. The episodes of fever and other symptoms typically become less frequent and severe over time.

Related Pages

• Aphthous stomatitis
• Pharyngitis
• Adenitis
• Cyclic neutropenia
• Familial Mediterranean fever
• Systemic juvenile idiopathic arthritis
• Corticosteroids
• Tonsillectomy
• Cimetidine