Familial cold autoinflammatory syndrome 1; FCAS1

Disease ID:1025
Name:Familial cold autoinflammatory syndrome 1; FCAS1
Associated with:1 target
3 immuno-relevant ligands
Synonyms
Familial cold urticaria
Database Links
OMIM: 120100
Orphanet: ORPHA47045

Targets

NLRP3

Ligands

Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
IL-1β
Immuno Disease Comments: Approved therapy for FCAS1.
canakinumab
Immuno Disease Comments: An anti-IL-1β therapeutic approved for FCAS1.
Clinical Use: Used to treat familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS) in patients > 4 years of age, and systemic juvenile idiopathic arthritis (SJIA) in patients > 2 years old. | View clinical data
rilonacept
Immuno Disease Comments: Approved drug for FCAS1.
Clinical Use: Currently used in the treatment of cryopyrin-associated periodic syndrome (CAPS). Cryopyrin-associated periodic syndrome includes three previously distinct disorders: Familial cold autoinflammatory syndrome; Muckle-Wells syndrome; and CINCA syndrome, that are now considered to represent a disease continuum, all caused by NLRP3 protein mutations.

EMA approval has been withdrawn. | View clinical data