Clinical Guidance

To date, more than 200 individuals have been identified with a pathogenic variant in TNFRSF1A . As a periodic fever syndrome, TNF receptor-associated periodic fever syndrome (TRAPS) is characterized by episodes of inflammation typically occurring every four to six weeks and lasting between five and 25 days. Flares may be prompted by stress, infection, trauma, hormonal changes, and vaccination. Symptoms may include fever, abdominal pain, arthralgia, myalgia, migratory rash, and eye inflammation, with variable severity. Symptoms often begin in early childhood (median age 4.3 years), though symptom onset can occur later in life, especially in those with mild or somatic pathogenic variants . During a flare, acute-phase reactants such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and serum amyloid A are typically elevated. Generally, acute-phase reactants stabilize between flares but may remain somewhat elevated even in the absence of clinical symptoms. Symptom severity does not appear to correlate with sex. Table 2. TNF Receptor-Associated Periodic Fever Syndrome: Frequency of Select Features

Diagnostic Criteria

Many clinical diagnostic criteria for TNF receptor-associated periodic fever syndrome (TRAPS) have been proposed. A classification system that incorporates molecular genetic testing and clinical features has been found to have a sensitivity of 95%, a specificity of 99%, and an accuracy of 99% .

Suggestive Findings

TRAPS should be considered in individuals with the following clinical, supportive laboratory, and family history findings.

Clinical findings

Source:

Management Guidelines

Clinical practice guidelines for the management of monogenic autoinflammatory conditions, including TNF receptor-associated periodic fever syndrome (TRAPS), have been published; see (full text; log in or purchase required) and (full text). Evaluations Following Initial Diagnosis To establish the extent of disease and needs in an individual diagnosed with TRAPS, the evaluations summarized (if not performed as part of the evaluation that led to the diagnosis) are recommended. Table 4. Recommended Evaluations Following Initial Diagnosis in Individuals with TNF Receptor-Associated Periodic Fever Syndrome

Surveillance Guidelines

In addition to a complete physical examination (performed annually or sooner if clinically indicated) with blood pressure and other vital signs, full skin examination, assessment for the presence of lymphadenopathy and hepatosplenomegaly, and musculoskeletal evaluation, the evaluations outlined in are recommended.

Table 6.

Recommended Surveillance for Individuals with TNF Receptor-Associated Periodic Fever Syndrome