Cryopyrin-Associated Periodic Syndromes (CAPS)

There are three main conditions within the CAPS spectrum, which vary in terms of severity:

Familial Cold Autoinflammatory Syndrome (FCAS): This is the mildest form of CAPS. Symptoms are triggered or worsened by exposure to cold temperatures. Patients experience rash, fever, joint pain, and other signs of inflammation during these episodes.

Muckle-Wells Syndrome (MWS): MWS is a moderate form of CAPS. Symptoms are more persistent and may not always be triggered by cold exposure. Patients with MWS experience recurrent fever, rash, joint pain, and eye redness. Progressive hearing loss can also occur.

Chronic Infantile Neurological, Cutaneous, and Articular Syndrome (CINCA) or Neonatal-Onset Multisystem Inflammatory Disease (NOMID): CINCA/NOMID is the most severe form of CAPS. Symptoms begin in infancy and include rash, joint swelling, chronic meningitis, and skeletal abnormalities. Neurological symptoms are also common, leading to developmental delays.

Symptoms & Characteristics

Common in All AiArthritis Diseases

Flares: Periods of worsening symptoms are called flares. A flare can last for hours, days, weeks, or months.

Physical Activity: Condition improves with activity and exercise and worsens with rest.

Comorbidities: When inflammation is left uncontrolled due to lack of proper treatment, comorbidities can develop. 70% of patients with chronic, lifelong disease will develop comorbidities, including dual or triple diagnoses.

Family History: Autoimmune diseases often run in families, indicating a potential genetic predisposition where that gene can cause disease. Autoinflammatory diseases can occur multiple times in a family, but is based off of genetic mutation. It is not a gene that causes the disease— but a mutation on the gene that can cause the disease which can then be passed on to the next generation.

"Auto" Symptoms

Fatigue: Severe fatigue or exhaustion that may not be helped by caffeine/stimulants and can happen even after a long period of rest.

Cognitive Dysfunction: Brain fog  or periods of time where thinking gets clouded and it becomes difficult to concentrate.

Flu-like symptoms: Without having the flu- nausea, muscle weakness, and general malaise.

Fever: Typically low grade in autoimmune (with exception of juvenile idiopathic arthritis) and higher grade in autoinflammatory (% strongly varies per disease).

Reference: Early Symptoms of AiArthritis Study, AiArthritis, 2019.

Inflammatory Arthritis Symptoms

Stiffness: Severe stiffness in one or more joints, especially in the morning or after sitting for long periods of time. 

Joint Pain: Episodes of joint pain that may last for hours, days, or even weeks, that can appear and disappear suddenly. Often described as “jumping pain” into different locations.

Typically the joint pain will coincide with one or more “Auto” symptoms and start and stop suddenly - for no apparent reason (which is called a "flare"). Some people will experience all of the above symptoms, others only a few.

 If you have any of the arthritis features, and at least one of the “Auto” features, please consult your physician about a referral to a specialist.

Symptoms Often Associated with CAPS

Familial Cold Autoinflammatory Syndrome (FCAS): Rash triggered by cold, muscle aches, eye redness

Muckle-Wells Syndrome (MWS): Skin lesions, cold sensitivity, amyloidosis, hearing loss

Chronic Infantile Neurological, Cutaneous, and Articular Syndrome (CINCA) or Neonatal-Onset Multisystem Inflammatory Disease (NOMID): Non-itchy hives, headaches, bulging eyes, organ inflammation, hearing loss, developmental delays

In an effort to ensure this page has the most accurate and up-to-date information, this page is currently awaiting medical review. Some information is subject to change.

Page Last Updated: 10/31/2024