About Cryopyrin-Associated Periodic Syndromes
Cryopyrin-Associated Periodic Syndromes represent a spectrum of autoinflammatory disorders caused by gain-of-function mutations in NLRP3 encoding cryopyrin, an inflammasome component that drives pathological IL-1 beta overproduction, ranging in severity from the mild cold-triggered urticaria of FCAS to the chronic devastating multisystem inflammation of NOMID/CINCA syndrome. TNF Receptor-Associated Periodic Syndrome (TRAPS) is caused by TNFRSF1A mutations and is included in this grouping as a related periodic fever syndrome with overlapping phenotypic features. IL-1 inhibition with anakinra, canakinumab, or rilonacept has transformed outcomes in CAPS, while TRAPS is primarily managed with IL-1 or TNF blockade.
Common Clinical Features
Clinical Trial Eligibility Tips
What to know before applying to Cryopyrin-Associated Periodic Syndromes trials.
Molecular confirmation of NLRP3 or TNFRSF1A pathogenic variant is required for most trials; somatic mosaic mutations may be present and require sensitive sequencing methods if standard testing is negative
Anakinra and canakinumab trials for CAPS typically require minimum C-reactive protein or SAA elevation at screening; ensure assessments are performed off anti-IL-1 therapy during washout
Pediatric and adult arms are often separate; confirm age cutoffs and weight thresholds (especially for somatic NOMID patients with growth delay)
Patient Resources
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