Immunodeficiency 97 With Autoinflammation C5676946
A rare condition combining immunodeficiency with abnormal inflammatory responses. Treatment aims to manage infections and control excessive inflammation through specialized immune-based therapies.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Recurrent fevers
Aseptic meningitis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Rash
(integumentary)Joint swelling
(musculoskeletal)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
Anti-inflammatory Therapy
Targeted immunomodulatory medications to control autoinflammatory manifestations including fever episodes, skin rashes, and systemic inflammation. Corticosteroids may be used initially for acute flares.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement
Regular immunoglobulin infusions to address the immunodeficiency component and provide immunological protection while anti-inflammatory therapies control autoinflammation.
Additional treatment options
Biologic Response Modifiers
Advanced biologic therapies such as IL-1 inhibitors or TNF-alpha blockers that specifically target inflammatory pathways involved in autoinflammatory disease manifestations.
medicationInflammatory Markers and Disease Activity Monitoring
Regular laboratory assessment including CRP, ESR, and cytokine levels to track disease activity and guide therapeutic adjustments. Imaging studies may assess organ involvement.
monitoringAnti-inflammatory Nutritional Support
Dietary modifications including omega-3 fatty acid supplementation and elimination of pro-inflammatory foods to potentially reduce baseline inflammatory burden. Adequate protein intake supports immune function.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Immunodeficiency 97 With Autoinflammation C5676946?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy