Tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS)
- Peter A Nigrovic, MD
Peter A Nigrovic, MD
- Associate Professor of Medicine
- Harvard Medical School
- Section Editors
- E Richard Stiehm, MD
E Richard Stiehm, MD
- Editor-in-Chief — Allergy and Immunology
- Section Editor — Immunology and Immunodeficiency
- Distinguished Research Professor of Pediatrics
- David Geffen School of Medicine at UCLA
- Sheldon L Kaplan, MD
Sheldon L Kaplan, MD
- Editor-in-Chief — Pediatrics
- Section Editor — Pediatric Infectious Diseases
- Professor and Vice Chairman for Clinical Affairs
- Baylor College of Medicine
Many well known disorders of immune excess result from a failure of self/nonself discrimination by T and B cells, the effector cells of the adaptive arm of the immune system. (See "Overview of autoimmunity".)
A second category of immune-mediated disease results from inappropriate activation of antigen-independent inflammatory mechanisms. This group of disorders has been termed "autoinflammatory diseases." An overview of autoinflammatory diseases is presented separately. (See "Periodic fever syndromes and other autoinflammatory diseases: An overview".)
Autoinflammatory diseases prominently involve cells of the innate immune system as well as the mediators associated with these cells. Thus, autoinflammatory diseases are broadly considered to represent primary diseases of innate immunity, in contrast to the autoimmune diseases that result from aberrant adaptive or acquired immunity. However, this separation is far from absolute.
The best characterized autoinflammatory diseases are relatively rare but florid conditions that arise from mutations in single genes. The prototypical autoinflammatory disorders are the periodic fever syndromes. One of these disorders, the tumor necrosis factor (TNF) receptor-1 associated periodic syndrome (TRAPS; MIM #142680, formerly known as familial Hibernian fever) is the subject of this topic review.
TRAPS is a rare disorder, with a prevalence of approximately one per million [1,2]. While originally described in an Irish (Hibernian) kindred, cases have since been reported in many ethnic groups. Thus, ancestry plays little role in the consideration of this disorder .
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- Grimwood C, Despert V, Jeru I, Hentgen V. On-demand treatment with anakinra: a treatment option for selected TRAPS patients. Rheumatology (Oxford) 2015; 54:1749.
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- CLINICAL MANIFESTATIONS
- Other features
- Secondary (AA) amyloidosis
- LABORATORY FINDINGS
- DIFFERENTIAL DIAGNOSIS
- Recurrent fevers due to bacterial and other infectious and neoplastic causes
- Connective tissue diseases
- Other autoinflammatory diseases
- - Familial Mediterranean fever
- - Hyperimmunoglobulin D syndrome
- - PFAPA syndrome
- - Cyclic neutropenia
- Antiinflammatory drugs
- Anti-TNF therapy
- SUMMARY AND RECOMMENDATIONS