Cryopyrin-Associated Periodic Syndromes and Treatment Options
Journal
Open Access Macedonian Journal of Medical Sciences
Date Issued
2020-09-03
Author(s)
Abstract
Cryopyrin-associated periodic syndromes (CAPSs) are a growing family of autoinflammatory diseases, also known
as periodic fever syndromes. There are three forms of CAPS: (1) Familial Cold autoinflammatory syndrome or familial
cold urticaria, (2) Muckle-wells syndrome, and (3) neonatal-onset multisystem inflammatory disease or chronic
infantile neurological cutaneous articular syndrome. Genetic mutations in the NLRP3 gene were found to be present
in most patients. The foremost common findings between all the CAPS disorders are rash, fever which is sometimes
present at birth or in early childhood, joint problems, and conjunctivitis. More extreme forms of CAPS include more
persistent inflammation that can cause hearing loss and meningitis and can lead to mental and developmental
delays. Drugs for CAPS target the source of inflammation – which is the over-production of interleukin 1ß by modified
cryopyrin inflammasomes. Three drugs are used to treat CAPS: Rilonacept, canakinumab, and anakirna. With these
drugs, the prognosis is greatly improved, with most patients having less frequent episodes, decreased buildup of
amyloid in the body, and extended life of severe cases up to adulthood.
as periodic fever syndromes. There are three forms of CAPS: (1) Familial Cold autoinflammatory syndrome or familial
cold urticaria, (2) Muckle-wells syndrome, and (3) neonatal-onset multisystem inflammatory disease or chronic
infantile neurological cutaneous articular syndrome. Genetic mutations in the NLRP3 gene were found to be present
in most patients. The foremost common findings between all the CAPS disorders are rash, fever which is sometimes
present at birth or in early childhood, joint problems, and conjunctivitis. More extreme forms of CAPS include more
persistent inflammation that can cause hearing loss and meningitis and can lead to mental and developmental
delays. Drugs for CAPS target the source of inflammation – which is the over-production of interleukin 1ß by modified
cryopyrin inflammasomes. Three drugs are used to treat CAPS: Rilonacept, canakinumab, and anakirna. With these
drugs, the prognosis is greatly improved, with most patients having less frequent episodes, decreased buildup of
amyloid in the body, and extended life of severe cases up to adulthood.
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