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Rare autoinflammatory and miscellaneous diseases 

Rare autoinflammatory and miscellaneous diseases
Rare autoinflammatory and miscellaneous diseases

Gavin Clunie

, Nick Wilkinson

, Elena Nikiphorou

, and Deepak R. Jadon

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date: 26 June 2022

This chapter provides a systematic approach to rare autoimmune and autoinflammatory or monogenetic genetic conditions. These conditions typically present with characteristic fevers such as familial Mediterranean fever, TRAPS, cryopyrin-associated periodic syndromes, and adult-onset Still’s disease; characteristic skin manifestations such as panniculitis, neutrophilic dermatoses, and eosinophilic fasciitis; or with refractory synovial disease or polychondritis. Rare multisystem conditions of unknown aetiology including sarcoidosis, Behçet’s disease, amyloidosis, and immunoglobulin G4-related disease are described in terms of organ or system involvement.

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