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Systemic diseases 

Systemic diseases
Chapter:
Systemic diseases
Author(s):

Brogan Foster

and Paul A. Brogan

DOI:
10.1093/med/9780198738756.003.0004
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date: 20 August 2019

This chapter covers the full spectrum of systemic diseases in paediatric rheumatology including: the systemic vasculitides (HSP, Kawasaki disease, PAN, ANCA-associated vasculitis, Takayasu arteritis, Behçet's disease, cerebral vasculitis, and many others); juvenile SLE; scleroderma; JDM; overlap syndromes; antiphospholipid syndrome; sarcoid; and paediatric uveitis. In addition, it provides updated descriptions and treatment approaches for autoinflammatory diseases, including recently described diseases such as DADA, SAVI, CANDLE, and many others. Other systemic diseases described in detail include mucopolysaccharidoses and mucolipidoses; musculoskeletal features of chromosomal abnormalities; cystic fibrosis; and inflammatory bowel disease. Treatment guidelines for all these systemic diseases have been fully updated, and aligned with recent evidence-based/consensus European guidance.

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