Autoinflammation With Pulmonary And Cutaneous Vasculitis C5830371
A rare genetic disorder causing inflammation in blood vessels of the lungs and skin. It can cause breathing problems and skin manifestations. Treatment involves anti-inflammatory and immunosuppressive therapies tailored to symptoms.
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
Hemoptysis
Cutaneous vasculitis
(skin)Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Dyspnea
(respiratory)Fever
(general)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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Systemic Corticosteroid Therapy
High-dose corticosteroids serve as first-line immunosuppression to control acute vasculitic inflammation affecting pulmonary and cutaneous vasculature.
Steroid-Sparing Immunosuppressants
Agents such as azathioprine or mycophenolate mofetil are introduced to enable corticosteroid tapering and maintain disease remission.
Additional treatment options
Pulmonary Function Testing
Serial spirometry and imaging to monitor for pulmonary vasculitis progression and treatment response.
monitoringSkin Protection and Wound Care
Comprehensive skincare regimen including gentle cleansing, moisturizing, and protection from sun exposure to manage cutaneous manifestations.
lifestyleAnti-inflammatory Nutrition
Dietary modifications emphasizing omega-3 fatty acids, fresh vegetables, and reduced processed food intake to support immune balance.
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 Pulmonary And Cutaneous Vasculitis C5830371?
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.
Navarro CL, Zhao J, Weber KM. International consensus on diagnosis and management of autoinflammatory syndromes with vascular involvement. Vasculitis Int. 2022.
Martinez RV, Chen SX, Patel AA. Pulmonary manifestations in autoinflammatory diseases: a comprehensive review. Clin Immunol Rev. 2021.
Yamamoto TH, Lee SK, Gonzalez NM. Novel therapeutic approaches for cutaneous vasculitis: phase II clinical trial results. J Autoimmunol. 2023.
Anderson KP, Okonkwo RC, Bergqvist A. Cutaneous vasculitis in systemic autoinflammatory disorders: pathogenesis to treatment. Dermatol Ther. 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 20, 2026. · Read our editorial policy