Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases

Table 2

Diagnostic criteria for patients with suspected cryopyrin-associated periodic syndrome (CAPS); adapted from Kuemmerle-Deschner et al. [98].

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Mandatory item

 Increase of inflammatory markers
 (C-reactive protein and/or serum amyloid A)

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Additional items

 (i) Urticaria-like rash
 (ii) Cold-triggered episodes
 (iii) Sensorineural hearing loss
 (iv) Musculoskeletal symptoms
 (v) Chronic aseptic meningitis
 (vi) Skeletal abnormalities

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At least 2 additional items are requested

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