. Author manuscript; available in PMC: 2021 Nov 8.

Published in final edited form as: J Clin Immunol. 2019 May 10;39(3):277–286. doi: 10.1007/s10875-019-00638-z

Table 1 –

Clinical features of CAPS

	FCAS	MWS	CINCA/NOMID
Cutaneous	Urticaria-like rash	Urticaria-like rash	Urticaria-like rash
Systemic	Fever / Fatigue / Chills	Fever / Fatigue	Fever / Fatigue
Musculoskeletal	Arthralgia / Myalgia	Arthralgia / Myalgia / Arthritis	Arthralgia / Myalgia / Distal femur overgrowth
Ocular	Conjunctivitis / Keratitis	Conjunctivitis/Keratitis/Uveitis	Conjunctivitis / Keratitis / Uveitis / Papillitis
Auditory		Sensorineural hearing loss	Sensorineural hearing loss
Central nervous system	Headache	Headache	Sterile meningitis, Elevated intracranial pressure
Morbidity	Amyloidosis (rare)	Amyloidosis	Amyloidosis, developmental delay
Episode Pattern	12–24 hours	1–3 days	Chronic with 1–3 day flares
Triggers	Generalized cold / pneumovax	Stress / exercise / infection / pneumovax	Stress / exercise / infection / pneumovax

Cryopyrin associated periodic syndrome (CAPS), Familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), Chronic infantile neurologic cutaneous articular (CINCA) syndrome or Neonatal onset multisystemic inflammatory disease (NOMID)