Immune

Cryopyrin-Associated Periodic Syndromes

Also known as CAPS, TRAPS, Muckle-Wells syndrome, NOMID

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, rangin

ORPHA:425 ↗Gene NLRP3Gene TNFRSF1APrevalence CAPS: 1-2 in 1,000,000; TRAPS: 1 in 1,000,000Onset Infancy to early childhood for CAPS; any age for TRAPSAutoinflammatory periodic fever syndrome (inflammasomopathy)

5

studies recruiting now

as of 7 Sept 2026

37

studies registered in total

as of 7 Sept 2026

4

countries with a recruiting site

as of 7 Sept 2026

25 Nov 2025

most recent study posted

among recruiting studies

Recruiting trials

RecruitingNCT06838143

Ilaris NIS in Korea

Sponsor Novartis PharmaceuticalsWhere South Korea (1 site)Studying IlarisUpdated 19 May 2026

Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.

Search all Cryopyrin-Associated Periodic Syndromes studies

Where recruiting studies are running

Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.

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Patient organisations

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Registry: Eurofever Registry · Join ↗. Registries connect patients to researchers and often hear about trials first.

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

Recurrent urticarial rash (non-pruritic, cold-triggered in FCAS)Fever and systemic inflammation during attacksSensorineural hearing loss (Muckle-Wells, NOMID)Chronic meningitis and elevated intracranial pressure (NOMID)Joint deformities and epiphyseal overgrowth (NOMID)Uveitis and papilledemaAA amyloidosis with renal involvement in untreated disease

From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.

Before you apply

Things trial teams commonly ask about for Cryopyrin-Associated Periodic Syndromes. Not eligibility rules; those are set by each study.

  • 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)

Related conditions

Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).