Autoinflammation And Autoimmunity With Immune Dysregulation 2 C6065928
A condition involving excessive immune activation causing recurrent fevers and autoimmune features. Typically presents in childhood. Treatment aims to control inflammation and modulate the immune system.
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
Systemic inflammation
Autoimmune cytopenias
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Dysregulated immune response
(immunologic)Urticarial 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
Immunomodulatory therapy
Systemic immunomodulatory agents to regulate abnormal immune responses and reduce autoinflammatory activity. Treatment selection based on specific immune dysregulation patterns identified through laboratory evaluation.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Comprehensive immune monitoring
Regular assessment of inflammatory markers, immune cell function, and autoantibody levels to track disease activity and treatment response over time.
Additional treatment options
Stress management and sleep optimization
Implementation of relaxation techniques, adequate sleep hygiene, and stress reduction strategies to minimize flare triggers and support immune balance.
lifestyleAnti-inflammatory nutritional support
Dietary modifications emphasizing omega-3 fatty acids, antioxidants, and elimination of pro-inflammatory foods to complement medical therapy.
dietaryAdjunctive complementary therapies
Selected complementary approaches such as acupuncture or guided imagery may provide supportive symptom management alongside conventional treatment.
complementaryWhen 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 Autoinflammation And Autoimmunity With Immune Dysregulation 2 C6065928?
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.
Castro CN, Son M, Bialer A. International consensus guidelines for autoinflammatory immune dysregulation syndromes. Clin Immunol. 2022.
Sanchez GAM, Lee HH, Craglia AF. Immune dysregulation in autoinflammatory syndromes: clinical features and pathogenesis. Nat Rev Rheumatol. 2021.
Koller RJ, Hoffmann GF, Kusters B. Targeted immunotherapy for immune dysregulation disorders: a phase II clinical trial. J Allergy Clin Immunol. 2023.
Rashid S, Bernthi R, Torres AR. Autoimmunity and autoinflammation with immune dysregulation: mechanisms and therapeutic approaches. Immunol Rev. 2020.
Medication Comparisons
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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.
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 27, 2026. · Read our editorial policy