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TRAPS ICD-10: E-85.0

Definition

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is one periodic fever syndrome / auto-inflammatory disease with periodic fever, red eye, rash, arthritis (arthritis) and stomach.

Occurrence

Rare. Disease start most often among children, less often among (young) adults

  • Variable debut age
  • Symptoms often begin at 3-4 years of age, but wide variation (1-11 years)
  • Disposable ethnicity: Iraq, Scotland and Mediterranean
  • Autosomal dominant inheritance
  • Mutation in the gene encoding the extracellular domain of 55kF TNF receptor = TNFRSF 1A mutation.

Symptoms

Fever: days

  • The symptoms usually last at least one week at a time
  • The number of fever episodes is 4-12 each year
  • Conjunctivitis (red eyes)
  • Periorbital edema (swollen eyes)
  • Abdominal pain (abdominal area)
  • Monoarthritis (one swollen joints)
  • Muscle Pain
    • Muscle pain may be located, migrating and be related to eczema or redness of the skin
  • Eczema: single or multiple spots with gradual spread down the arms and legs
  • Increased blood test (CRP), blood drop reaction (SR)
  • Biopsy (tissue test): Deep infiltration of lymphocytes and monocytes (white blood cells). Monocytic fasciitis may explain myalgia (not myocyte)

Diagnosis

Exclude infection and cancer (malignancy)

  • Disease history
    • Duration of episodes
      • Rash
      • Eyes
      • Periorbital Infection (More than 80%)
      • Muscle pain (focal) in almost everyone
      • Often similar symptoms in close family
    • Ensure the diagnosis of genetic testing: TNFR1 gene. Some carriers are without symptoms (asymptomatic)
    • Consider screening (gentest) by close relatives

Criteria

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

Genetic test showing TNFRSF1A genotype and at least one of the following:

  • Duration of fever at least 7 days
  • Muscle pain
  • Moving rash (migrant)
  • Periorbital edema (Swelling around the eyes)
  • Relatives with the same disease

OR

Absence of genetic test showing TNFRSF1A genotype and at least two of the following:

  • Duration of fever at least 7 days
  • Muscle pain
  • Moving rash (migrant)
  • Periorbital edema (Swelling around the eyes)
  • Relatives with the same disease

Suggested criteria for diagnosis and classification (Federci, S, PRINTO / Eurofever 2015)

Occurrence Points

  • Periorbital edema 21
  • Duration of episodes> 6 days 19
  • Migrants characteristic rash 18
  • Myalgia 6
  • Relatives with the same disease 7
  • Absence of vomiting 14
  • Absence of aphthous stomatitis 15

TOTAL score at least 43

Sensitivity 59%, Specificity 84%

Incorrect diagnosis (similar conditions, differential diagnosis)

Complication

AA amyloidosis occurs later in the course of the disease

Treatment

NSAIDs, corticosteroids (prednisolone), etanercept (Not infliximab), anakinra (Kineret), canacunimab (Ilaris). Somewhat useful colchicine.

Literature

Auto-inflammatory diseases (BINDEVEVSSYKDOMMER.no)


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