Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is a condition causing recurring fevers, primarily affecting children. It is not contagious and is considered benign, posing no long-term danger to physical health. The syndrome is characterized by its predictable symptom pattern, distinguishing it from other conditions.
Understanding PFAPA
PFAPA is an autoinflammatory disorder, differing from autoimmune diseases or infections. In autoinflammatory conditions, the innate immune system becomes dysregulated, leading to inflammation without an external trigger like a virus or bacteria. Its exact cause is unknown, but it involves abnormal immune activation causing fever and inflammation.
The syndrome is marked by recurrent fevers with notable periodicity, often every 3 to 6 weeks. Children are completely well between episodes. This predictability helps distinguish PFAPA from other fever conditions.
Recognizing PFAPA Symptoms
The name PFAPA describes its core symptoms: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis. High fevers are a primary symptom, lasting between 3 to 7 days. Aphthous stomatitis refers to small, painful mouth sores, while pharyngitis indicates a sore throat, sometimes with white patches on the tonsils. Cervical adenitis involves swollen lymph nodes in the neck.
Children experiencing PFAPA episodes may also have headache, abdominal pain, joint pain, rash, vomiting, or diarrhea. Episodes start abruptly, and the child may appear ill during an attack.
Diagnosing PFAPA
Diagnosing PFAPA is a process of exclusion, meaning other recurrent fever causes must be ruled out. Doctors base diagnosis on the recurring symptom pattern and a physical examination. Clinical criteria include regular fever periodicity, characteristic symptoms (aphthous stomatitis, pharyngitis, and cervical adenitis), and the absence of other conditions explaining fevers.
No specific blood tests confirm PFAPA, but tests exclude other conditions, like infections or other autoinflammatory diseases. During a fever episode, inflammation markers like C-reactive protein and erythrocyte sedimentation rate may be elevated, but these return to normal between episodes. A prompt, positive response to corticosteroid treatment also supports a PFAPA diagnosis.
Treating PFAPA
The primary treatment for PFAPA focuses on managing acute episodes and reducing their frequency. A single dose of corticosteroids, such as prednisone, often aborts fever episodes and shortens their duration. While corticosteroids are highly effective for acute management, their repeated use might lead to more frequent episodes in some cases.
Supportive care, including pain relievers and fever reducers, enhances comfort during an episode. For some, preventative strategies may be considered. Tonsillectomy, the surgical removal of the tonsils, can resolve the syndrome in some children. Other options, like cimetidine, may also be used preventatively.
Living with PFAPA
PFAPA is a benign condition with a favorable long-term outlook. The syndrome resolves spontaneously as children grow, often by late childhood or early adolescence. Children with PFAPA experience normal growth and development and do not incur long-term organ damage. Although episodes can significantly impact a child’s quality of life due to missed school days and discomfort, consistent support from family and medical professionals is important in managing the syndrome’s episodic nature.