PFAPA 4/5 (1)

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Periodic fever with aphthous stomatitis, pharyngitis and adenitis (PFAPA)

PFAPA

Periodic pain and redness in the throat with subsequent regular periods of fever in children and adolescents may be PFAPA syndrome. Dake / Wikimedia.org. CC BY 2,5

Definition

PFAPA syndrome is probably the most common condition  fever syndrome / auto-inflammatory diseases, but the cause is probably more complex than mutation in a single gene, as in most other periodic fever syndromes. Typically, young children become ill with a high fever without any infection being detected or antibiotics (Penicillin and others) having an effect. Fever episodes last up to 5 days and recur at regular intervals, often monthly. Many people can eventually predict, almost during the day, when the next fever attack will come. No genetic test is available (as of 2019).

Disease Cause

Unlike most other types fever syndrome / auto-inflammatory disease PFAPA is probably not caused by a single gene defect and no gene tests are available.

Symptoms

Symptom debut most often in children at 1-5's age

  • Periods with fever every 3-4 week (periodic fever)
  • The fever lasts up to 5 days
  • Often without symptoms from special organs
    • Sore throat
    • Mouth ulcers
    • Lymph node swellings
    • Exhaustion
    • Headache
    • Some stomach ache
  • The child is in good shape between the periods of fever and grows normally

Diagnosis

Typical medical history with at least three episodes of fever that lasts up to five days and returns periodically.

  • Sore and red throat that may have sores or blisters
  • Good shape between fever attacks
  • Exclusion of other fever cause (infections, Leukemia or other types of cancer)
  • Blood tests
    • CRP and ESR rise during fever. Neutrophilic leukocytes (white blood cells) and platelets (platelets) may also increase. Normalization between the fever episodes

Classification criteria Eurofever / PRINO 2019 (Gattorno M, 2019)

The criteria are primarily intended for research, so that the diagnosis can be made on another basis in clinical practice.

At least seven of the following eight items must be present:

  1. Pharyngo-tonsillitis (inflammation of the throat and tonsils)
  2. Duration of episodes 3-6 days
  3. Swollen lymph nodes on the neck
  4. Periodic symptoms (asymptomatic between seizures)

Furthermore absence of the following:

  1. Diarrhea
  2. Chest pain
  3. Rash
  4. Arthritis (inflamed joint)

Treatment

Prednisone can be used during fever attacks. Cimetidine can also be used. Some have an effect of removing tonsils (toncillectomy). Off label treatment is IL-1 blocks (Kineret, Ilaris) that may have effect (reference: Stojanov S, 2011).

Medical prognosis

Most people "grow the disease on their own" without lasting damage by the end of adolescence

Literature

Auto-inflammatory diseases (BINDEVEVSSYKDOMMER.no)


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