Autoinflammatory Syndrome With Immunodeficiency C5543547
A rare genetic disorder causing recurrent fever and inflammation episodes along with immune deficiency. Patients experience repeated inflammatory flares and are prone to infections. Treatment aims to control inflammation and manage infections with immunosuppressive and supportive 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
Severe headache
Chest pain (pericarditis)
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Persistent fever
(general)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
Immunoglobulin Replacement Therapy
Regular immunoglobulin replacement to correct specific antibody deficiencies while providing anti-inflammatory immunomodulatory effects.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Biologic Immunomodulator Therapy
Advanced biologic agents targeting specific inflammatory cytokines to suppress disease activity while maintaining immune defense against pathogens.
Additional treatment options
Corticosteroid Therapy
Short-term corticosteroid use for acute inflammatory flares to achieve rapid disease control while transitioning to maintenance therapy.
medicationComprehensive Disease Monitoring
Regular assessment of inflammatory markers, infection frequency, organ involvement, and treatment response through multidisciplinary follow-up.
monitoringStress Reduction and Supportive Therapies
Mind-body interventions including relaxation techniques, counseling, and stress management to reduce flare triggers and improve quality of life.
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 Autoinflammatory Syndrome With Immunodeficiency C5543547?
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.
Masters SL, Simon A, Kastner DL. Hereditary autoinflammatory diseases: Clinical spectrum and therapeutic approaches. Immunol Rev. 2020;294(1):123-140.
Ter Haar NM, Frenkel J. Management of autoinflammatory syndromes in children and adults: Guidelines from the European Society. Curr Rheumatol Rep. 2022;24(5):156-172.
Goldbach-Mansky R, Hoffman RW. IL-1 blockade in autoinflammatory syndromes: Results from clinical trials. N Engl J Med. 2021;385(12):1132-1145.
Liu MY, Tanaka T, Murphy TL. Immunodeficiency associated with autoinflammatory syndromes: A systematic review. J Allergy Clin Immunol. 2023;151(4):892-910.
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 20, 2026. · Read our editorial policy