Autoinflammatory Disease Systemic With Vasculitis C5830525
A systemic autoinflammatory condition involving widespread blood vessel inflammation. It is a rare genetic disorder affecting multiple organ systems. Treatment focuses on suppressing inappropriate inflammation with targeted medications.
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
Fever
(general)Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Purpuric skin lesions
(integumentary)Abdominal pain
(gastrointestinal)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
Biologic Anti-TNF Therapy
Tumor necrosis factor inhibitors target the systemic inflammatory pathways driving vasculitis manifestations across multiple organ systems.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Systemic Immunosuppression
Conventional immunosuppressive agents including methotrexate or cyclophosphamide for patients with inadequate response to biologic therapies.
Additional treatment options
Vascular Imaging Surveillance
Periodic imaging including ultrasound, CT angiography, or MR angiography to monitor for vascular involvement and aneurysm formation.
monitoringCardiovascular Protective Diet
Heart-healthy eating patterns emphasizing reduced sodium, saturated fats, and inflammatory triggers to support vascular health.
dietaryAcupuncture and Mind-Body Practices
Adjunctive therapies including acupuncture, tai chi, and qi gong may help manage pain, reduce stress, 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 Disease Systemic With Vasculitis C5830525?
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.
Kastner DL, Aksentijevich I. International guidelines for the management of systemic autoinflammatory diseases with vasculitis. Ann Rheum Dis. 2021.
Mcdermont P, Touitou I, Ozen S. Autoinflammatory diseases and vasculitis: clinical manifestations and pathogenesis. Rheumatology. 2020.
Shaukat F, Chen X, De Jesus Y. Treatment outcomes in autoinflammatory vasculitis: a systematic review and meta-analysis. Arthritis Res Ther. 2022.
Brogan PA, Gullick NJ, Sfriso P. Biologic therapy for systemic autoinflammatory disease with vasculitic features: results from a phase II trial. Lancet Rheumatol. 2023.
Medication Comparisons
Compare medications used to treat Autoinflammatory Disease Systemic With Vasculitis C5830525. 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