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ICD10 code of Muckle-Wells Syndrome and ICD9 code

What is the ICD10 code for Muckle-Wells Syndrome? And the ICD9 code for Muckle-Wells Syndrome?

ICD9 and ICD10 codes of Muckle-Wells Syndrome

Muckle-Wells Syndrome is a rare autoinflammatory disorder characterized by recurrent fever, rash, joint pain, and hearing loss. The ICD-10 code for Muckle-Wells Syndrome is M04.2. Unfortunately, there is no specific ICD-9 code for this syndrome as it was replaced by ICD-10. It is important to consult with a healthcare professional for accurate diagnosis and appropriate treatment.


Muckle-Wells Syndrome (MWS) is a rare hereditary autoinflammatory disorder characterized by recurrent episodes of urticaria (hives), fever, joint pain, and other systemic symptoms. It falls under a group of conditions known as cryopyrin-associated periodic syndromes (CAPS).

In the International Classification of Diseases, 10th Revision (ICD-10), the specific code for Muckle-Wells Syndrome is M04.2. This code is primarily used to identify and classify the condition for administrative and billing purposes. It helps medical professionals and insurers to accurately document and track the prevalence and incidence of this syndrome.

On the other hand, in the previous version, the International Classification of Diseases, 9th Revision (ICD-9), there is no specific code for Muckle-Wells Syndrome. However, it would typically fall under the broader category of "Other specified rheumatoid arthritis and other inflammatory polyarthropathies" with the code 714.8. This code is used to encompass various inflammatory joint conditions that are not explicitly listed under specific codes.

It is important to note that the transition from ICD-9 to ICD-10 occurred on October 1, 2015, in most countries. This change aimed to provide a more comprehensive and detailed classification system for medical conditions, allowing for better accuracy in diagnosis and treatment.

In summary, the ICD-10 code for Muckle-Wells Syndrome is M04.2, while in ICD-9, it would typically fall under the broader code 714.8. These codes assist healthcare professionals in accurately categorizing and documenting the condition for administrative and clinical purposes.
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2 answers
ICD-10-CM M04.2 Cryopyrin associated periodic syndrome, CINCA, Familial Cold Uticaria, Muckle Wells Syndrome
ICD-9- CM 710.8

Posted Apr 26, 2018 by Angelina 1850

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