The Indian Journal of Pediatrics

, Volume 77, Issue 10, pp 1173–1181 | Cite as

Fevers and the Rheumatologist

Symposium on Pediatric Rheumatology-II

Abstract

Fevers in children are mainly due to infection, malignancy or inflammatory conditions. Rheumatologists have an important role in the care of inflammatory conditions, many of which are associated with fevers. Seven conditions, the hereditary recurrent fever syndromes, have been defined with the presenting symptom of recurring fever, and for which mutation of a single gene has been defined: Chronic infantile neurological articular syndrome (CINCA), Familial cold autoinflammatory syndrome (FACS), Familial Mediterranean fever (FMF), hyperimmunoglobulinemia D (HIDS), Muckle-Wells syndrome (MWS), Pyogenic sterile arthritis and Pyoderma gangrenosum (PAPA) and Tumour necrosis factor receptor-associated periodic syndrome (TRAPS) . These conditions will be discussed in detail in regard to how they fit into the wider picture of pediatric rheumatological conditions, how the diagnoses may be established and the current recommended treatments for each condition.

Keywords

Hereditary recurrent fever syndromes Cryopyrinopathies 

Abbreviations

CINCA syndrome

Chronic Infantile Neurological Articular Syndrome

FCAS

Familial Cold Autoinflammatory Syndrome

FMF

Familial Mediterranean Fever

HIDS

Hyperimmunoglobulinemia D Syndrome

MWS

Muckle-Wells Syndrome

NOMID

Neonatal-Onset Multi-System Inflammatory Disease

PAPA

Pyogenic Sterile Arthritis, Pyoderma Gangrenosum and Acne

PFAPA

Periodic Fever, Aphthous Ulcers, Pharyngitis and Cervical Adenitis

TRAPS

Tumour Necrosis Factor Receptor-Associated Periodic Syndrome

SLE

Systemic Lupus Erythematosus

JIA

Juvenile Idiopathic Arthritis

DM

Dermatomyositis

MCTD

Mixed Connective Tissue Disease

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Copyright information

© Dr. K C Chaudhuri Foundation 2010

Authors and Affiliations

  1. 1.School of Paediatrics and Child HealthUniversity of Western Australia, c/- Princess Margaret Hospital for ChildrenPerthAustralia

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