Short answer · Medically reviewed summary · Last updated: 2026-05-08
Hyper-IgD Syndrome (HIDS), also known as Mevalonate Kinase Deficiency (MKD), is classified under the ICD-10-CM code E88.89 (Other specified metabolic disorders) and the older ICD-9-CM code 277.6 (Other disorders of enzyme metabolism). Because HIDS is a rare autoinflammatory condition, it does not have a unique, disease-specific code in either system, which often complicates insurance authorization and clinical tracking. What is the clinical significance of HIDS? Hyper-IgD Syndrome is a rare autoinflammatory disorder caused by mutations in the MVK gene, leading to a deficiency of the enzyme mevalonate kinase.
ICD10 code of Hyper-IgD Syndrome / Hyperimmunoglobulinemia D With Recurrent Fever (HIDS) and ICD9 code
ICD-10 and ICD-9 codes for Hyper-IgD Syndrome / Hyperimmunoglobulinemia D With Recurrent Fever (HIDS), with classification details for clinicians, coders and patients.
Hyper-IgD Syndrome (HIDS), also known as Mevalonate Kinase Deficiency (MKD), is classified under the ICD-10-CM code E88.89 (Other specified metabolic disorders) and the older ICD-9-CM code 277.6 (Other disorders of enzyme metabolism). Because HIDS is a rare autoinflammatory condition, it does not have a unique, disease-specific code in either system, which often complicates insurance authorization and clinical tracking.
What is the clinical significance of HIDS?
Hyper-IgD Syndrome is a rare autoinflammatory disorder caused by mutations in the MVK gene, leading to a deficiency of the enzyme mevalonate kinase. Patients with Hyper-IgD Syndrome typically experience recurrent episodes of fever lasting 3 to 7 days, often accompanied by lymphadenopathy, arthralgia, abdominal pain, and skin rashes. While the ICD-10 code E88.89 is used for billing, clinical management focuses on systemic inflammation control.
How is Hyper-IgD Syndrome diagnosed?
Diagnosis of Hyper-IgD Syndrome involves a combination of clinical evaluation and laboratory testing. Physicians often look for elevated levels of IgD in the blood, though this is not present in all patients. Definitive diagnosis is confirmed through genetic testing for MVK mutations and the measurement of mevalonic acid levels in the urine during or between fever episodes.
Key considerations for HIDS patients
- Inheritance: Hyper-IgD Syndrome is inherited in an autosomal recessive pattern, meaning both parents must carry a mutation.
- Prevalence: It is an ultra-rare disease with fewer than 300 cases documented globally in medical literature, though many more may remain undiagnosed.
- Community: At DiseaseMaps.org, 6 people with Hyper-IgD Syndrome have joined our community to share their experiences and navigate the challenges of this rare diagnosis.
- Treatment: Current management often involves IL-1 inhibitors to reduce the frequency and severity of inflammatory flares.
Next steps
- Consult with a rheumatologist or an immunologist who specializes in autoinflammatory diseases.
- Request genetic counseling to understand the inheritance pattern of Hyper-IgD Syndrome for your family.
- Connect with the 6 members of the Hyper-IgD Syndrome community at DiseaseMaps.org to share management strategies.
- Monitor your symptoms in a journal to help your doctor identify specific triggers for your fever episodes.
Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- Orphanet: Mevalonate kinase deficiency (ORPHA:419)
- NIH GARD: Hyperimmunoglobulinemia D with recurrent fever
- OMIM: Mevalonate Kinase Deficiency (MIM: 610377)
- Auto-Inflammatory Alliance: HIDS/MKD Patient Resources
