Table 2

Clinical characteristics of 136 cryopyrin-associated periodic syndrome patients

n%
Disease pattern
 Recurrent5843
 Chronic with exacerbations2518
 Chronic5339
Fever10884
 Constitutional symptoms*9570
Skin involvement13297
 Urticarial rash12189
 Maculopapular rash3626
 No rash43
Musculoskeletal involvement11786
 Myalgia6044
 Arthralgia10779
 Arthritis4736
 Severe musculoskeletal involvement†64
Neurological involvement5540
 Morning headache 3929
 Papilloedema2927
 Meningitis2326
 Severe neurological involvement‡1612
Ophthalmologic involvement9771
 Conjunctivitis8766
 Uveitis97
 Severe ophthalmological involvement§ 1612
Hearing loss5642
AA amyloidosis54
  • *Constitutional symptoms were defined as fever, fatigue, malaise, mood disorders or failure to thrive.

  • †Severe musculoskeletal involvement was defined as any of the following: joint contracture, patellar overgrowth, bone deformity, bone erosion or osteolytic lesion.

  • ‡Severe neurological involvement was defined as any of the following: seizure, hydrocephalus or mental retardation.

  • §Severe ophthalmological involvement was defined as any of the following: optic nerve atrophy, cataract, glaucoma or impaired vision.