Tumor Necrosis Factor (TNF) - Associated Periodic Syndrome – aka Familial Hibernian Fever

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Acronym:
TRAPS

SAID group:
Protein Folding

Gene:
TNFRSF1A

Inheritance:
Autosomal dominant. Spontaneous mutations, with some familial groups. [1]

Ethnicity:
Affects all races. Second most common inherited SAID (after FMF). [1]

Frequency:
Unknown. TRAPS affects 0.01:10,000 people in the European Union. [51] >1000 patients worldwide. [52]

Timing of symptoms:
Days to weeks. An average flare lasts around three weeks. [1] [9]

Age of onset:
Most first attacks occur by 3 years of age, and almost all begin by 20 years of age. A few have symptoms start later in life. [9]

Skin cutaneous:
Migrating rash with deep pain under the areas with the rash. Severe pain follows the rash path from the trunk outwards to the limbs. [9]

Neurologic:
Fevers lasting >3 days at over 38°C with flares. Some have headaches with flares of symptoms. [1] [9]

Auditory:
Uncommon–not believed to be caused by TRAPS. [1]

Ophthalamic:
Conjunctivitis, and periorbital edema during flares. [1] [9]

Cardiopulmonary:
Common, including pleurisy. [1]

Abdominal:
Abdominal pain, peritonitis, diarrhea, and constipation with flares. Splenomegaly. [1]

Lymphatic:
Splenomegaly is common; some have lymphadeopathy. [1]

Joints bones muscles cartilage:
Intermittent or chronic arthritis in the large joints with muscle pain and swelling is common. [1]

Vasculitis:
Henoch-Schönlein purpura (HSP), lymphocytic vasculitis. [1]

Amyloidosis:
10-20% occurrence. Higher risk with a cysteine mutation. [9]

Abnormal labs:
High: ESR, CRP, SAA. Polymorphonuclear neutrophils (PMNs), polyclonalgammopathy, leukocytosis. [1] Elevated serum IgD levels10% to 13% of patients with TRAPS (elevated in some with other autoinflammatory diseases too, such as HIDS, MA, FMF, PFAPA) [128]

Search Keywords:
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Photo credit:

Federici, S., Caorsi, R., & Gattorno, M. (2012). The autoinflammatory diseases. Swiss medical weekly, 142, 0.

Resources:

TRAPS Syndrome information on autoinflammatie.nl

Our Autoinflammatory Alliance website TRAPS Syndrome page.

TRAPS information in Spanish at autoinflamatorias.com

Find periodic fever syndrome panels, which test for TRAPS and syndromes with similar symptoms at the same time, at the Genetic Testing Registry (GTR).

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