jueves, 12 de abril de 2012

Patient Education - PFAPA (Pediatric) ► Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)

Patient Education - PFAPA (Pediatric)

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)

This syndrome includes recurrent episodes of fever with aphthous stomatitis (mouth sores) and pharyngitis (sore throat with redness). Occasionally, there also may be exudate (white patches on the tonsils) and enlarged lymph nodes in the neck (adenitis). Episodes of fever start suddenly and last for 3-7 days. Fevers occur routinely every few weeks; often, families know the exact day when an attack will start. Some children have other symptoms like joint pain, abdominal pain, headache, vomiting or diarrhea. Children are completely well between attacks.
The disease may last for several years but usually will resolve by itself after the child turns 10. Over time, the time between the attacks will increase. Children with PFAPA continue to grow and develop normally.
  • PFAPA is a syndrome that consists of recurrent attacks of fever, sore throat, mouth sores and swelling of the glands in the neck.
  • Use of steroids at the start of an episode can stop it, but also may shorten the time to the next episode.
  • PFAPA usually resolves spontaneously after age 10 years.
  • Tonsillectomy may cure the disease.
The frequency of PFAPA is not known, but the disease appears to be more common than originally thought, and may be the most common recurrent fever syndrome that does not come from an infection. Both males and females and all ethnic groups can develop PFAPA. PFAPA usually starts in early childhood, between the ages of 2 to 4 years.
The answer to this question is not yet known. No gene defect has yet to be found in PFAPA, although sometimes more than one family member has the disease. No infection  has been found in PFAPA, and it is not a contagious disease. It is clear that the inflammatory process is active during episodes, but it is not clear why this happens.
There are no laboratory tests specific for diagnosing PFAPA. The disease is diagnosed based on symptoms and physical examination. White blood cell counts, sedimentation rate and the C-reactive protein, all of which can be measured with a blood test, increase during attacks. It is important to exclude all other diseases that may present with similar symptoms (especially a Streptococcus infection) before confirming the diagnosis. The dramatic response to treatment also helps diagnose PFAPA.
The aim of the treatment will be to control symptoms during the attacks of fever, to shorten the duration of the attacks, and to prevent attacks from occurring. The fever usually does not respond well to acetaminophen (Tylenol) or nonsteroidal anti-inflammatory drugs like ibuprofen (Advil or Motrin). A single dose of steroids (usually prednisone), given when the symptoms first start, has been shown to shorten—and often even end—the attack. However, the time between attacks also may be shortened with this treatment, and the next attack may occur earlier than expected. Several studies have found that a tonsillectomy (removing the tonsils by surgery) cures PFAPA in the majority of patients (more than 80%).
Attacks may affect the quality of life of the child and the family and result in many missed days of school. There is no danger to the long-term health of the child.
  • PFAPA is a periodic fever syndrome that includes symptoms of pharyngitis, mouth sores and swelling of the neck glands.
  • Episodes usually end by age 10.
  • Steroids given at the start of an episode usually end it, but the result may be more frequent attacks.
  • Removing the tonsils in children with many attacks may cure the disease.
For a listing of pediatric rheumatologists in your area, click here.  Learn more about rheumatologists and rheumatology health professionals.
The American College of Rheumatology has compiled this list to give you a starting point for your own additional research. The ACR does not endorse or maintain these websites, and is not responsible for any information or claims provided on them. It is always best to talk with your rheumatologist for more information and before making any decisions about your care.
Created April 2011
Written by Phil Hashkes, MD, Hebrew University, Jerusalem, and reviewed by the American College of Rheumatology Special Committee on Pediatric Rheumatology.
This patient fact sheet is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnoses and treatment of a medical or health condition.
© 2011 American College of Rheumatology

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