Short answer · Medically reviewed summary · Last updated: 2026-05-08
Hyper-IgD Syndrome (HIDS), also known as Mevalonate Kinase Deficiency (MKD), is a rare autoinflammatory disorder primarily identified by recurrent fevers and elevated immunoglobulin D levels. It is officially classified under the broader spectrum of Mevalonate Kinase Deficiency, which encompasses both the milder HIDS phenotype and the more severe Mevalonic Aciduria. What are the common names and synonyms for HIDS? In medical literature, you will encounter several names for this condition, reflecting its evolution from a symptomatic description to a genetic understanding.
Hyper-IgD Syndrome / Hyperimmunoglobulinemia D With Recurrent Fever (HIDS) synonyms
Other names for Hyper-IgD Syndrome / Hyperimmunoglobulinemia D With Recurrent Fever (HIDS): synonyms, acronyms and related terms used by doctors and patients.
Hyper-IgD Syndrome (HIDS), also known as Mevalonate Kinase Deficiency (MKD), is a rare autoinflammatory disorder primarily identified by recurrent fevers and elevated immunoglobulin D levels. It is officially classified under the broader spectrum of Mevalonate Kinase Deficiency, which encompasses both the milder HIDS phenotype and the more severe Mevalonic Aciduria.
What are the common names and synonyms for HIDS?
In medical literature, you will encounter several names for this condition, reflecting its evolution from a symptomatic description to a genetic understanding. The most common synonyms include:
- Hyperimmunoglobulinemia D with recurrent fever (HIDS)
- Mevalonate Kinase Deficiency (MKD)
- Mevalonic Aciduria (referring to the most severe end of the disease spectrum)
- Dutch Type Periodic Fever (a historical name reflecting the region where many early cases were documented)
Why does this condition have multiple names?
The naming of Hyper-IgD Syndrome has evolved as scientific understanding shifted. Initially, the disease was named for its most prominent clinical feature: high levels of IgD in the blood. However, once researchers identified that the underlying cause was a deficiency in the enzyme mevalonate kinase, the term Mevalonate Kinase Deficiency (MKD) was adopted to cover the entire clinical spectrum. Today, clinicians prefer Mevalonate Kinase Deficiency as it describes the genetic root cause rather than just one biomarker.
How is this condition classified in medical databases?
Standardized classification systems use specific codes to ensure consistent identification of Hyper-IgD Syndrome across international borders:
- OMIM (Online Mendelian Inheritance in Man): #260920
- Orphanet: ORPHA406 (Mevalonate kinase deficiency)
- ICD-10: E88.89 (Other specified metabolic disorders)
Which name should I use when speaking with specialists?
While Hyper-IgD Syndrome remains widely recognized, referring to it as Mevalonate Kinase Deficiency is now considered the most accurate clinical terminology. If you are communicating with healthcare providers, using the term MKD helps bridge the gap between older diagnostic labels and current genetic classifications. Our DiseaseMaps.org community currently supports 6 members navigating life with Hyper-IgD Syndrome, sharing experiences across both historical and modern nomenclature.
Next steps
- Consult with a clinical geneticist to discuss the MVK gene mutation analysis.
- Join our global community at DiseaseMaps.org to connect with others managing Hyper-IgD Syndrome.
- Keep a detailed fever log to present to your rheumatologist or immunologist during your next appointment.
Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of a qualified physician regarding any medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Mevalonate kinase deficiency
- Orphanet: Mevalonate kinase deficiency (ORPHA406)
- OMIM: Mevalonate Kinase Deficiency (#260920)
- Autoinflammatory Alliance: Patient resources for MKD/HIDS
