Table 6.

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

System/ConcernEvaluationFrequency
Rheumatologic
  • Measure acute-phase reactants incl CRP, ESR, serum level of AA amyloid, fibrinogen, haptoglobin, & CBC w/differential. 1
  • Assess w/Autoinflammatory Diseases Activity Index (AIDAI). 2
Annually or more frequently if indicated
Gastrointestinal
  • Liver function tests 3
  • Imaging of abdomen to assess for splenomegaly/hepatomegaly may also be considered if there are findings of serum AA amyloid or suspected spleen or liver enlargement on physical exam.
Renal Measurement of renal function 4 & urinalysis 5
Eyes Ophthalmologic exam for ptosis, abnormal eye movements, retinal infarcts, optic nerve damage
Infectious/
Immunologic 		Measure quantitative serum immunoglobulins.
Screen for tuberculosis, typically by QuantiFERON® measurement.For any affected person on biologic treatment, as risk of infection may be ↑

CBC = complete blood count; CRP = C-reactive protein; ESR = erythrocyte sedimentation rate; MOI = mode of inheritance; TRAPS = TNF receptor-associated periodic fever syndrome

1.

To assess for leukocytosis and elevated neutrophil count, thrombocytosis, and normochromic anemia, which may be related to secondary chronic inflammation.

2.

This checklist reviews 12 symptoms and provides an assessment of whether an affected individual is experiencing frequent disease activity that may require optimization of the treatment regimen or if the affected individual is fairly well controlled in terms of their autoimmune symptoms.

3.

Liver function testing may include assessment of serum aspartate aminotransferase, alanine transaminase, protein, and albumin.

4.

Renal function testing may include serum blood urea nitrogen, creatinine, cystatin-C, and electrolytes.

5.

To assess for proteinuria

From: TNF Receptor-Associated Periodic Fever Syndrome

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