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AutoimmuneICD-10: I77.6Varies by type; overall estimated at 20-50 per million per year for systemic vasculitides

Vasculitis

Vasculitis is a group of disorders characterized by inflammation of the blood vessels, which can cause the vessel walls to thicken, narrow, weaken, or scar, leading to restricted blood flow and damage to affected organs. Vasculitis can affect any type of blood vessel—arteries, veins, or capillaries—and any organ system, making it an extremely variable disease. The condition may be primary (occurring on its own) or secondary to other conditions such as infections, autoimmune diseases, or medications.

Symptoms

Fever, fatigue, and general malaise
Unexplained weight loss
Muscle and joint pain
Skin changes including purpura (purple or red spots), ulcers, or nodules
Numbness, tingling, or weakness in the hands or feet (peripheral neuropathy)
Headaches, vision changes, or scalp tenderness (in giant cell arteritis)
Cough, shortness of breath, or coughing up blood (pulmonary involvement)
Blood in the urine or declining kidney function (renal involvement)

Causes

  • Autoimmune attack on blood vessel walls (most common mechanism)
  • Infections triggering immune-mediated vessel inflammation (hepatitis B, hepatitis C, HIV)
  • Medications causing hypersensitivity vasculitis
  • Other autoimmune diseases such as lupus, rheumatoid arthritis, or scleroderma
  • The exact cause is unknown in many primary vasculitis types

Risk Factors

  • Existing autoimmune disease
  • Chronic hepatitis B or C infection
  • Smoking (particularly for Buerger's disease)
  • Age and sex vary by type (giant cell arteritis affects those over 50; Kawasaki disease affects young children)
  • Certain medications or drug use (cocaine-associated vasculitis)

Diagnosis

  • Blood tests including ESR, CRP, CBC, and comprehensive metabolic panel
  • Autoantibody testing (ANCA, ANA, complement levels, cryoglobulins)
  • Tissue biopsy of affected organs (skin, kidney, nerve, temporal artery) for histological confirmation
  • Angiography (CTA, MRA, or conventional angiography) to detect vessel narrowing or aneurysms
  • Urinalysis to assess kidney involvement
  • Imaging studies (CT, MRI, PET scan) to evaluate organ involvement

Treatment

  • High-dose corticosteroids (prednisone) for initial inflammation control
  • Immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, mycophenolate) for remission induction and maintenance
  • Biologic therapies (rituximab) for ANCA-associated vasculitis
  • Plasma exchange (plasmapheresis) for severe or rapidly progressive disease
  • Treatment of underlying infections (antivirals for hepatitis-associated vasculitis)
  • Organ-specific supportive care (dialysis for kidney failure, wound care for skin ulcers)

Prevention

  • No known prevention for most primary vasculitis types
  • Treat underlying infections (hepatitis B, C) to prevent secondary vasculitis
  • Quit smoking, particularly to prevent Buerger's disease
  • Avoid known drug triggers when possible
  • Regular monitoring and early treatment of flares can prevent organ damage

When to See a Doctor

  • You develop unexplained fever, weight loss, and fatigue with skin rashes or joint pain
  • You notice purpura (non-blanching purple spots), skin ulcers, or new numbness/weakness
  • You experience vision changes, severe headaches, or scalp tenderness (urgent evaluation needed for giant cell arteritis)

Frequently Asked Questions

Related Conditions

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High Blood Pressure

Cardiovascular

Anemia

Metabolic

Stroke

Cardiovascular

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Medical Disclaimer: This content is for educational and informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare provider.

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