Hyperimmunoglobulin D With Periodic Fever C0398691
Hyperimmunoglobulin D with periodic fever is an inflammatory condition causing recurring fever episodes and elevated IgD levels. It often begins in childhood. Most people manage symptoms effectively with treatment.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent fever episodes
(immunologic)Abdominal pain
(gastrointestinal)Joint pain
(musculoskeletal)Skin rash
(integumentary)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Non-steroidal anti-inflammatory drugs
NSAIDs help manage fever and inflammatory symptoms during acute attacks. They work by reducing prostaglandin synthesis, which contributes to the inflammatory response in HIDS.
Colchicine
Colchicine is an anti-inflammatory agent that can reduce the frequency and severity of febrile episodes in some HIDS patients by inhibiting neutrophil migration and activity.
Additional treatment options
IL-1 blocking agents
Biologic agents like anakinra or canakinumab target interleukin-1 signaling, addressing the underlying autoinflammatory mechanism of HIDS when conventional therapies are insufficient.
medicationCorticosteroids
Systemic corticosteroids may be prescribed for severe flares that do not respond to NSAIDs or colchicine, providing potent anti-inflammatory effects.
medicationDisease activity monitoring
Regular clinical assessment and tracking of fever episodes, inflammatory markers, and complications helps guide treatment adjustments and prognosis discussions.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Hyperimmunoglobulin D With Periodic Fever C0398691?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
van der Hilst JC, Simon A, Drenth JP, et al. International consensus recommendations for the diagnosis and management of hyperimmunoglobulin D syndrome. Orphanet J Rare Dis. 2020.
Shendi HM, Edgar JD, McCluskey D, et al. Hyperimmunoglobulin D syndrome in children: clinical features and treatment outcomes. Clin Rheumatol. 2019.
Bodar EJ, van der Meer JW, Simon A. Anakinra for hyper-IgD syndrome: a prospective open-label study. J Inherit Metab Dis. 2018.
Rigante D, Cantarini L, Lucherini OM, et al. Advances in understanding and management of mevalonate kinase deficiency and hyper-IgD syndrome. Expert Rev Clin Immunol. 2016.
Medication Comparisons
Compare medications used to treat Hyperimmunoglobulin D With Periodic Fever C0398691. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy