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What is the prevalence of Muckle-Wells Syndrome?

How many people does Muckle-Wells Syndrome affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Muckle-Wells Syndrome

Muckle-Wells Syndrome is a rare autosomal dominant disorder characterized by recurrent episodes of fever, rash, joint pain, and other systemic symptoms. It is caused by mutations in the NLRP3 gene. The prevalence of Muckle-Wells Syndrome is estimated to be around 1 in 1,000,000 individuals worldwide. Due to its rarity, the syndrome is considered to be a very uncommon condition. Early diagnosis and appropriate management are crucial in improving the quality of life for individuals affected by this syndrome.



Muckle-Wells Syndrome (MWS) is a rare genetic disorder that falls under the category of autoinflammatory diseases. It is characterized by recurrent episodes of fever, rash, joint pain, and other systemic symptoms. MWS is caused by mutations in the NLRP3 gene, which leads to overactivation of the immune system.

The prevalence of Muckle-Wells Syndrome is estimated to be very low, with only a few hundred cases reported worldwide. Due to its rarity, it is considered an orphan disease. The exact prevalence rate is difficult to determine accurately, as many cases may go undiagnosed or misdiagnosed. However, it is believed to affect less than 1 in 1 million individuals.

MWS is typically diagnosed in childhood or early adulthood, and its symptoms can vary in severity among affected individuals. Without proper management, the disease can lead to progressive hearing loss, kidney damage, and other complications.

While there is currently no cure for Muckle-Wells Syndrome, treatment options are available to help manage the symptoms and improve the quality of life for affected individuals. Early diagnosis and appropriate medical intervention are crucial in minimizing the impact of this rare disorder.
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2 answers
The incidence of CAPS has been reported to be approximately 1 in 1,000,000 people in the United States and Europe. The genetic mutation is passed on for generations in families. There is a 50% chance that a parent with CAPS or a genetic mutation will be pass it along to his or her child.

Posted Apr 26, 2018 by Angelina 1850

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