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(Pediatrics in Review. 2009;30:e34-e41.)
© 2009 American Academy of Pediatrics

Periodic Fever Syndromes

Abbreviations: CAPS: cryopyrin-associated periodic syndromes • CINCA: chronic infantile neurologic cutaneous and articular syndrome • ESR: erythrocyte sedimentation rate • FCAS: familial cold autoinflammatory syndrome • FMF: familial Mediterranean fever • HIDS: hyperimmunoglobulin D syndrome • Ig: immunoglobulin • IL: interleukin • MVK: mevalonate kinase • MWS: Muckle-Wells syndrome • NOMID: neonatal-onset multisystem inflammatory disease • NSAID: nonsteroidal anti-inflammatory drug • PFAPA: periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome • TNF: tumor necrosis factor • TRAPS: tumor necrosis factor receptor-associated periodic syndrome

The first 300 words of the full text of this article appear below.

	Objectives

 
After completing this article, readers should be able to:

1. Describe the differences between the periodic fever (or autoinflammatory syndromes) and autoimmune disorders.
   
2. Summarize the ethnic predilection and genetic basis for the most common periodic fever syndromes.
   
3. Recognize the salient clinical features of the most common periodic fever syndromes
   
4. List the current therapeutic choices for the management of the most common periodic fever syndromes.
   

	Introduction

 
Any list of the causes of fever of unknown origin in childhood is extensive and must include broad categories such as infectious diseases, neoplastic conditions, and rheumatic/inflammatory disorders. The periodic fever syndromes most often have been included in the rheumatic/inflammatory category, but their proper classification and pathogenesis remain unclear. Within the past 10 years, significant strides have been made in the understanding of the periodic fever syndromes, which now are considered to result from primary dysregulation of the innate immune system.

Many rheumatic disorders that are considered when evaluating for a cause of fever of unknown origin are defined by aberrations of the adaptive or acquired immune system. Antigen recognition by adaptive immune surveillance is accomplished through T and B lymphocytes and is characterized by the enhanced development of either autoantibodies or autoreactive T cells.

Within the innate immune system, aberrations are not based on self-targeting by autoantibodies or lymphocytes but rather occur through the activation of antigen-independent inflammatory mechanisms. Neutrophils, macrophages, and natural killer cells (rather than T and B cells) and tumor necrosis factor (TNF), interleukin-1 (IL-1), and IL-12 are the primary cellular effectors and mediators of innate immunity.

To accommodate this new information, the term autoinflammatory disorders has been advanced to classify these conditions more accurately. Many of these disorders are hereditary and have typical ethnic predilections. Thus, the periodic fever syndromes from the nosologic view now are considered to exist within the broader . . . [Full Text of this Article]

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