PFAPA Syndrome (Juvenile): Symptoms, Treatment

Overview

PFAPA syndrome or periodic fever with aphthous stomatitis, pharyngitis, adenitis is a chronic and complex autoinflammatory disease. It is characterized by episodes of high fever occurring about every 3-5 weeks, accompanied with a sore throat, mouth sores and swelling of the glands in the neck.

PFAPA is often first-noted in children under 5 years old. Occasionally it may develop at an older age, including rare cases in adults. Both males and females from all ethnic groups can develop the disease.

Cause

We do not know what causes PFAPA. For unexplained reasons, the body’s immune system becomes active and attacks its own cells and tissues, leading to the symptoms of the disorder.

According to the latest information, no gene or infection play a role in developing the disease. It is not a contagious disease.

Symptoms

The main signs and symptoms of PFAPA are those in its name, including

  • Periodic fever lasting for 3-7 days at intervals of about 3–5 weeks
  • Aphthous stomatitis, meaning mouth sores
  • Pharyngitis, referring to the sore throat with redness
  • Adenitis, indicating swelling of the glands

Other manifestations that PFAPA may cause include:

  • Joint pain
  • Skin rash
  • Abdominal pain
  • Headache
  • Vomiting
  • Diarrhea

These attacks eventually stop by late childhood. There are no long-term effects. Children with PFAPA can grow and develop normally.

Diagnosis

There are no laboratory tests or imaging tests specific to the diagnosis of PFAPA. Doctors may confirm the diagnosis mainly based on your symptoms. For individuals who have a history of 3 or more episodes of fevers that last up to 5 days and recur at regular intervals, and if they do not have other evidence of acute illness, chances are that they have PFAPA.

Blood tests like white blood cell count, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) are also helpful in diagnosing the condition. It is also important to rule out other conditions that may present with similar symptoms. The dramatic response to treatment with steroids may also help diagnose PFAPA.

Treatment

PFAPA syndrome typically resolves spontaneously. Treatment options are used to control symptoms during the episodes of fever, to shorten the duration of the episodes, and to prevent episodes from occurring. Treatment is either medical or surgical.

There is no medication that can cure PFAPA. Effective medications include:

Surgical removal of the tonsils and adenoids appears to be useful. Although it is not clear why the surgery works.


Keywords: periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome; PFAPA.

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* The Content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.