Muckle-Wells Syndrome (NLRP3-AID-moderate)

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MWS (NLRP3-AID-moderate)

SAID group:
Cryopyrin Associated Periodic Syndromes (CAPS) NLRP3-associated Autoinflammatory Disease (NLRP3-AID)


Autosomal dominant. Spontaneous mutations, and some family groups with MWS spanning generations. [1]

Affects all races, but many are of European descent. [1]

1:1 million, but it may be more frequent. Some large family groups. [5] Frequency of CAPS in France is 1:360,000. [55]

Timing of symptoms:
Often lasts 2-3 days. Random onset–flares of fever and symptoms are often triggered by cold or cooling temperature. [1]

Age of onset:
Infancy, but a few present with symptoms later in childhood or adolescence. [1]

Skin cutaneous:
Urticaria-like rash with increased neutrophils at the eccrine coils. Rash and flares can be cold-induced, or from unknown triggers. [4] Most have a daily rash that increases with flares. [1] A few have apthous ulcers. Negative ice cube test.

Some have headaches, fatigue with fever and flares. It is uncommon to have many other central nervous system symptoms. [1] A few patients have a MWS/NOMID crossover of symptoms.

Many have increased sensorineural hearing loss, starting in adolescence. [1]

Conjunctivitis (non-infectious) during flares,[1] or corneal haze. [26] MWS/NOMID crossover patients may have more eye involvement.

Rare. [1]

Some have abdominal pain with flares or other gastrointestinal issues. [1]

Rarely noted. [1]

Joints bones muscles cartilage:
Arthralgia, recurrent arthritis, stiffness and swelling with flares. [1]

Not noted. [1]

Elevated serum amyloid (SAA). >25 % with secondary amyloidosis. [1], [9]

Abnormal labs:
High: ESR, CRP, SAA. Leukocytosis with flares. [1]

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    Find periodic fever syndrome panels, which test for CAPS and syndromes with similar symptoms at the same time, at the Genetic Testing Registry (GTR).

    Download or view our CAPS Guidebook in English, or choose "ES" at the top of the page to access the Spanish version.

    Our Autoinflammatory Alliance website Muckle-Wells page.

    Find periodic fever syndrome panels, which test for CAPS and syndromes with similar symptoms at the same time, at the Genetic Testing Registry (GTR).

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