Short answer · Medically reviewed summary · Last updated: 2026-05-08
Muckle-Wells Syndrome is categorized under the ICD-10 code E85.0 (Hereditary familial amyloidosis) and the ICD-9 code 277.30 (Amyloidosis, unspecified). As a rare autoinflammatory condition, Muckle-Wells Syndrome requires precise coding to ensure appropriate clinical management and insurance coverage for systemic treatment. What is Muckle-Wells Syndrome? Muckle-Wells Syndrome is a rare autoinflammatory disorder belonging to the Cryopyrin-Associated Periodic Syndromes (CAPS) spectrum.
1 people with Muckle-Wells Syndrome have shared their first-person experience on this question at DiseaseMaps.
ICD10 code of Muckle-Wells Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Muckle-Wells Syndrome, with classification details for clinicians, coders and patients.
Muckle-Wells Syndrome is categorized under the ICD-10 code E85.0 (Hereditary familial amyloidosis) and the ICD-9 code 277.30 (Amyloidosis, unspecified). As a rare autoinflammatory condition, Muckle-Wells Syndrome requires precise coding to ensure appropriate clinical management and insurance coverage for systemic treatment.
What is Muckle-Wells Syndrome?
Muckle-Wells Syndrome is a rare autoinflammatory disorder belonging to the Cryopyrin-Associated Periodic Syndromes (CAPS) spectrum. It is characterized by recurrent episodes of fever, urticaria-like skin rashes, and joint pain. Because Muckle-Wells Syndrome involves the overproduction of interleukin-1 beta (IL-1β), it can lead to serious long-term complications, most notably secondary amyloidosis, which may result in progressive kidney damage if left untreated.
How is Muckle-Wells Syndrome diagnosed?
Diagnosis of Muckle-Wells Syndrome is primarily clinical, supported by genetic testing and laboratory markers of inflammation. Physicians typically look for the following diagnostic indicators:
- Elevated acute-phase reactants, such as C-reactive protein (CRP) and serum amyloid A (SAA).
- Genetic confirmation of a mutation in the NLRP3 gene.
- Clinical history of cold-induced urticaria and progressive sensorineural hearing loss.
- Persistent systemic inflammation even during periods without fever.
Is Muckle-Wells Syndrome hereditary?
Yes, Muckle-Wells Syndrome is an autosomal dominant condition, meaning an affected individual has a 50% chance of passing the NLRP3 gene mutation to each offspring. However, it is important to note that some patients with Muckle-Wells Syndrome have a de novo mutation, meaning it occurred spontaneously without a family history of the disorder.
Living with Muckle-Wells Syndrome
Our community at DiseaseMaps.org currently includes 15 members navigating life with Muckle-Wells Syndrome. Connecting with others can provide essential emotional support, as the chronic nature of Muckle-Wells Syndrome can feel isolating. Managing the condition often involves IL-1 blocking therapies, which have significantly improved the prognosis for patients.
Next steps
- Consult with a rheumatologist or immunologist specializing in autoinflammatory diseases.
- Request a genetic consultation to discuss family planning and inheritance patterns.
- Join the DiseaseMaps.org community to share experiences with others living with Muckle-Wells Syndrome.
- Monitor renal function regularly through urine and blood tests to screen for amyloidosis.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment.
References
- Orphanet: Muckle-Wells Syndrome (ORPHA:2482)
- NIH GARD: Cryopyrin-Associated Periodic Syndromes
- OMIM: NLRP3-Related Autoinflammatory Disease (#191900)
- Autoinflammatory Alliance: Patient resources for CAPS
ICD-9- CM 710.8
Posted Apr 26, 2018 by Angelina 1850
