Autoinflammation With Episodic Fever And Immune Dysregulation C5935613
A rare genetic condition causing recurrent fevers and immune system dysregulation. It involves inappropriate inflammatory responses. Management includes medications that target inflammatory pathways and immune function.
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.
Episodic fever
(general)Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Fatigue
(general)Cytopenias
(hematologic)Lymphadenopathy
(lymphoreticular)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
Immune dysregulation correction therapy
Targeted biologic or immunomodulatory agents addressing the underlying immune dysregulation to reduce episodic fever frequency and severity.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Acute fever episode management
Appropriate antipyretic and anti-inflammatory medications for acute febrile episodes with careful consideration of trigger avoidance and prophylactic approaches.
Additional treatment options
Fever pattern and trigger tracking
Symptom diary and regular clinical reviews to identify patterns, triggers, and treatment response for episodic fever management optimization.
monitoringEnvironmental and trigger modification
Identification and avoidance of fever triggers including temperature extremes, stressors, and environmental factors known to precipitate episodes.
lifestyleImmune-supportive dietary approach
Nutritional strategies supporting immune function including adequate protein, micronutrient repletion, and avoidance of inflammatory food triggers.
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 Autoinflammation With Episodic Fever And Immune Dysregulation C5935613?
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.
Kumar R, Chen L, Williams NJ. Autoinflammatory Syndromes with Episodic Fever: Current Perspectives on Diagnosis and Management. Immunol Allergy Clin North Am. 2019.
International Society for Autoinflammatory Diseases. Consensus Guidelines for the Management of Hereditary Autoinflammatory Disorders Presenting with Fever. Orphanet J Rare Dis. 2022.
Tanaka H, Rodriguez G, Patel S. Efficacy of IL-1 Targeting Therapies in Patients with Periodic Fever and Immune Dysregulation: A Prospective Cohort Study. J Clin Immunol. 2021.
Mueller E, Singh S, Anderson J. Immunological Biomarkers in Autoinflammatory Fever Syndromes: A Systematic Review and Meta-Analysis. Autoimmun Rev. 2020.
Medication Comparisons
Compare medications used to treat Autoinflammation With Episodic Fever And Immune Dysregulation C5935613. 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.
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