Cryopyrin-associated periodic syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Overview
Cryopyrin-associated periodic syndrome (CAPS) is a rare disease entity encompassing three different clinical phenotypes including Muckle-Wells syndrome (MWS), neonatal-onset multisystem inflammatory disorder (NOMID), and familial cold autoinflammatory syndrome (FCAS). The three aforementioned phenotypes occur due to a common gene mutation.
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Xyz from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
An elevated/reduced concentration of serum acute-phase reactant is diagnostic of cryopyrin-associated periodic syndrome.
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
There are no other imaging findings associated with the cryopyrin-associated periodic syndrome.
Other Diagnostic Studies
There are no other diagnostic studies associated with cryopyrin-associated periodic syndrome.
Treatment
Medical Therapy
There is no definitive treatment for cryopyrin-associated periodic syndrome; the mainstay of therapy is supportive care. Supportive therapy for cryopyrin-associated periodic syndrome includes anakinra, rilonacept, and canakinumab. Patients with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab. Symptomatic treatment options include nonsteroidal anti-inflammatory drugs, disease-modifying antirheumatic drugs, and glucocorticoids.
Surgery
Surgical intervention is not recommended for the management of the cryopyrin-associated periodic syndrome.
Primary Prevention
There are no established measures for the primary prevention of cryopyrin-associated periodic syndrome.
Secondary Prevention
There are no established measures for the secondary prevention of cryopyrin-associated periodic syndrome.