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ICD10 code of Cryopyrin-associated periodic syndrome and ICD9 code

What is the ICD10 code for Cryopyrin-associated periodic syndrome? And the ICD9 code for Cryopyrin-associated periodic syndrome?

ICD9 and ICD10 codes of Cryopyrin-associated periodic syndrome

Cryopyrin-associated periodic syndrome (CAPS) is a rare autoinflammatory disorder characterized by recurrent episodes of fever, rash, joint pain, and other systemic symptoms. The ICD-10 code for CAPS is M04.2. Unfortunately, there is no specific ICD-9 code for CAPS as it was replaced by ICD-10 in 2015. CAPS is caused by mutations in the NLRP3 gene and can lead to severe complications if left untreated.


Cryopyrin-associated periodic syndrome (CAPS) is a rare autoinflammatory disorder characterized by recurrent episodes of inflammation. The condition is caused by mutations in the NLRP3 gene, leading to overproduction of the protein interleukin-1β (IL-1β), which triggers inflammation in the body.

In the International Classification of Diseases, Tenth Revision (ICD-10), Cryopyrin-associated periodic syndrome is classified under "Other specified systemic involvement of connective tissue" with the code M35.8. This code encompasses various other connective tissue disorders that do not have specific codes assigned to them.

On the other hand, the International Classification of Diseases, Ninth Revision (ICD-9) does not have a specific code for Cryopyrin-associated periodic syndrome since it was introduced after the transition to ICD-10. However, in ICD-9, similar autoinflammatory disorders such as familial Mediterranean fever (277.31) or systemic autoinflammatory diseases (277.39) could have been used to classify certain symptoms or manifestations associated with CAPS.

It is important to note that ICD codes are primarily used for billing and statistical purposes in healthcare settings. They serve as a standardized way to classify and categorize diseases, disorders, and other health conditions. These codes aid in medical record-keeping, research, and data analysis. Therefore, having a specific code for CAPS in ICD-10 allows healthcare professionals to accurately document and track the prevalence and impact of this rare condition.

Overall, Cryopyrin-associated periodic syndrome is classified under ICD-10 code M35.8, while ICD-9 does not have a specific code for this condition.
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ICD9 and ICD10 codes of Cryopyrin-associated periodic syndrome

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Stories of Cryopyrin-associated periodic syndrome

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Cryopyrin-associated periodic syndrome stories
I was born in the UK, and suffered (as did my Father) from undiagnosed FCAS for 3-+ years. Intense pain and rash was just referred to as "my wierd disease".   Back in about 2002 I was travelling on business in North Carolina, USA when I developed ...

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