What is PFAPA Syndrome?
PFAPA Syndrome stands for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis. It is a rare, non-infectious inflammatory disorder primarily affecting young children and characterized by recurrent episodes of fever along with mouth sores, sore throat, and swollen lymph nodes.
What causes PFAPA Syndrome?
The exact cause of PFAPA syndrome is unknown, but it is believed to involve an abnormal immune system response. It is not contagious or caused by infections. Genetic factors may play a role, though it is not typically inherited.
What are the symptoms of PFAPA Syndrome?
Symptoms usually start in early childhood and include:
- Sudden high fever lasting 3-6 days recurring every 3-8 weeks
- Painful mouth sores (aphthous stomatitis)
- Sore throat (pharyngitis) without bacterial infection
- Swollen and tender lymph nodes in the neck (adenitis)
- Fatigue and irritability during episodes
- Occasionally headache or abdominal pain
How is PFAPA Syndrome diagnosed?
Diagnosis is clinical, based on the pattern of symptoms and exclusion of other causes:
- Recurrent periodic fever episodes with consistent symptoms
- No evidence of infection during episodes
- Normal growth and development between episodes
- Blood tests may show elevated inflammatory markers during fever
- Sometimes genetic testing to rule out other periodic fever syndromes
How is PFAPA Syndrome treated?
Treatment focuses on symptom relief and reducing episode frequency:
- Corticosteroids like a single dose of prednisone or prednisolone often relieve symptoms quickly during attacks
- Tonsillectomy (removal of tonsils) may reduce or prevent episodes in some children
- Supportive care including fever reducers and hydration
- Most children outgrow the syndrome by adolescence
What is the prognosis for PFAPA Syndrome?
- Generally excellent; most children recover completely by their teenage years
- Episodes usually decrease in frequency and severity over time
- No long-term complications or lasting damage
How can parents manage PFAPA Syndrome?
- Keep a detailed symptom diary to track fever episodes
- Provide supportive care during fever episodes (rest, fluids, fever medication)
- Consult healthcare providers for corticosteroid treatment during attacks
- Regular follow-ups with a pediatrician or specialist to monitor condition
- Educate caregivers and teachers about the condition to ensure proper support