Immunodeficiency 72 With Autoinflammation C5436540
A rare condition combining immunodeficiency with autoinflammatory features, causing both immune weakness and periodic inflammation.
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
Aseptic meningitis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent fevers
(immunologic)Autoinflammatory skin lesions
(immunologic)Inflammatory bowel disease
(immunologic)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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin infusions to provide passive immunity and compensate for the immunodeficiency component of the disorder. This addresses the underlying antibody deficiency while supporting immune function.
Anti-inflammatory and Immunomodulatory Therapy
Targeted anti-inflammatory medications to manage the autoinflammatory manifestations of the condition. These agents help control inflammatory flares and prevent tissue damage while maintaining immune vigilance against infections.
Additional treatment options
Prophylactic Antibiotics
Continuous or intermittent antibiotic prophylaxis to prevent bacterial infections, particularly respiratory pathogens. Selection based on patient history and local resistance patterns.
medicationInfection Prevention Practices
Comprehensive lifestyle modifications including rigorous hand hygiene, respiratory precautions, and avoidance of crowded environments during high-risk periods. Education for patient and family on early infection recognition.
lifestyleRegular Immunological and Inflammatory Monitoring
Scheduled laboratory assessments including immunoglobulin levels, inflammatory markers, and immune cell function to guide treatment adjustments and predict flares.
monitoringWhen 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 72 With Autoinflammation C5436540?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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