FEVER CONUNDRUM
PFAPA syndrome, which was first described by Marshal and friends at 1987, is a syndrome mostly seen under the age of 5, including relapsing fever, oral aphtous lesions (mouth sores), pharyngitis and lumps on the neck. The fever is episodic, meaning in every 21-28 days (every month, often families know the exact day when an attack will start!), a 39oC fever occurs and lasts for 3-6 days. It can be accompanied by a sore throat, mouth ulcers, or enlarged cervical lymph nodes. During episodes, the child looks very ill and complains about at least one of the three symptoms mentioned above. On the day the fever starts, the child will feel a little bit ill before the attack and the family knows an attack is about to start. Not all children have all symptoms, especially mouth sores. Some children have other symptoms like joint pain, abdominal pain, headache, vomiting or diarrhea.
The answer to what causes this syndrome is not yet known. No gene defect has yet to be found in PFAPA, although in some cases more than one family member has the disease. No infectious cause has been found in PFAPA, thus it is not a contagious disease. It is clear that the inflammatory process is activated during episodes, but it is not clear why it is triggered.
There are no laboratory tests, or imaging procedures, specific for diagnosing PFAPA. The disease is diagnosed based on the results of a physical examination and other symptoms. Inflammatory blood tests like the white blood cell count, erythrocyte sedimentation rate and the C-reactive protein are increased during attacks. Before the diagnosis is confirmed, it is important to exclude all other diseases that may present with similar symptoms (especially a streptococcal throat). The dramatic response to treatment (see below) also helps diagnose PFAPA.
Beacuse the cause is unknown, there is no specific treatment to cure PFAPA. The aim of treatment is to control symptoms during the episodes of fever. In most children, the disease will resolve by itself without treatment, usually after the age of 10 years. A single dose of steroid (usually prednisone), given when symptoms first appear, has been shown to shorten an episode and sometimes even end the episode. However, the interval between episodes may also be shortened with this treatment, and the next episode may occur earlier than expected. In patients with very frequent attacks, a tonsillectomy (removing the tonsils by surgery) may be considered.
Over time, the intervals between the episodes will increase and usually after the age of 10 years resolve by itself. Children with PFAPA continue to grow and develop normally.
REFERENCES:
1. PFAPA Sendromu: Bir Periyodik Ateş Tablosu, Fırat Tıp Dergisi 2006;11(1): 75-77,
Metehan ÖZEN, Gül YÜCEL, İnönü Üniversitesi Tıp Fakültesi, Malatya
2.http://my.clevelandclinic.org/disorders/periodic_fever_syndrome/rheumatology_overview.aspx
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