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

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Acronym:
TRAPS

SAID group:
TNF-associated Autoinflammatory Diseases, 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]

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