Vasculitis

All Diseases

Introduction

Vasculitis is inflammation of blood vessel walls that can narrow, weaken, or block vessels supplying skin, nerves, kidneys, lungs, and other organs. Presentation varies from mild rash to life-threatening organ failure.

Forms may be primary autoimmune disorders or secondary to infection, medication, or other diseases. Diagnosis combines clinical pattern, laboratory markers, imaging, and often tissue biopsy.

Treatment usually requires immunosuppression tailored to disease severity and type, with close monitoring for relapse and medication toxicity.

This page explains common symptoms, how evaluation usually proceeds, treatment options, and when to seek medical care.

Overview

Large-vessel vasculitis includes giant cell arteritis and Takayasu arteritis. Medium-vessel disease includes polyarteritis nodosa and Kawasaki disease. Small-vessel disease includes ANCA-associated vasculitis and IgA vasculitis.

Early recognition prevents irreversible organ damage such as vision loss in giant cell arteritis or kidney failure in glomerulonephritis.

  • Inflammation of arterial or venous walls
  • Many subtypes by vessel size and organ pattern
  • Systemic symptoms: fever, fatigue, weight loss
  • ANCA testing and biopsy aid diagnosis
  • Corticosteroids are cornerstone of treatment
  • Relapse requires long-term monitoring

What happens in the body

Immune complexes or autoreactive leukocytes attack vessel walls, causing necrosis, aneurysm formation, or luminal stenosis. Complement activation and neutrophil infiltration dominate in ANCA-associated disease.

Ischemia of downstream tissue produces organ-specific symptoms. Chronic inflammation leads to fibrosis and stenosis in some large-vessel vasculitides.

  • Autoimmune attack on vascular endothelium
  • Neutrophil degranulation in ANCA vasculitis
  • Granulomatous inflammation in giant cell arteritis
  • Immune complex deposition in IgA vasculitis

Signs and symptoms

Common symptoms and warning signs may include:

  • Fever, malaise, and weight loss
  • New headache and jaw claudication (giant cell arteritis)
  • Skin purpura or ulcers
  • Joint and muscle pain
  • Sinuses, lung, or kidney involvement: cough, hemoptysis, blood in urine
  • Mononeuritis multiplex: wrist or foot drop
  • Abdominal pain from mesenteric ischemia
  • Hypertension in renal artery involvement

Causes and risk factors

Possible causes and contributing factors include:

  • Primary autoimmune dysregulation
  • Hepatitis B associated polyarteritis nodosa
  • Medication-induced ANCA vasculitis
  • Infection-triggered IgA vasculitis in children
  • Genetic susceptibility with environmental triggers
  • Associated connective tissue disease overlap
  • Sometimes idiopathic without clear trigger

Diagnosis and evaluation

Evaluation may include:

  • ESR and CRP often elevated
  • ANCA serology (PR3 and MPO)
  • Urinalysis and creatinine for renal involvement
  • Temporal artery or affected organ biopsy
  • Angiography or vascular imaging for stenosis or aneurysm
  • Chest imaging and pulmonary function tests
  • Exclude infection and malignancy mimics

Treatment and management

Treatment and management may involve:

  • High-dose corticosteroids for induction
  • Cyclophosphamide, rituximab, or methotrexate for severe disease
  • Plasma exchange in selected severe ANCA cases
  • Maintenance azathioprine or mycophenolate to prevent relapse
  • Treat hepatitis B when associated with vasculitis
  • Low-dose aspirin in giant cell arteritis when no contraindication
  • Supportive organ-specific care (dialysis, wound care)
  • Gradual steroid taper with relapse monitoring

Prevention, self-care, and lifestyle

Not every condition is fully preventable, but the steps below may lower risk or recurrence:

  • No general prevention for primary vasculitis
  • Prompt treatment of hepatitis B in endemic settings
  • Report new symptoms during biologic therapy

Possible complications

Delay, missed care, or unsafe self-medication can raise the chance of complications in some cases:

  • Permanent vision loss
  • End-stage kidney disease
  • Stroke from large-vessel stenosis
  • Bowel infarction
  • Infection from immunosuppression

When to see a doctor or seek emergency care

Seek prompt medical advice or emergency care if any of the following apply—it is safer not to wait and see:

  • New headache with visual changes in older adults
  • Blood in urine or rapidly rising creatinine
  • Chest pain, hemoptysis, or sudden neurologic deficit
  • Persistent fever and rash unresponsive to antibiotics

Living with the condition

Keep scheduled lab monitoring during immunosuppressive therapy and report fever or new symptoms early. Many people achieve remission but need years of follow-up because relapse can occur.

Frequently asked questions

Is vasculitis contagious?

Primary autoimmune vasculitis is not contagious. Some forms may be triggered by infections, but the inflammatory vessel disease itself does not spread person to person.

How long is treatment needed?

Induction therapy lasts months; maintenance often continues one to two years or longer depending on subtype and relapse risk.

Important caution

This article is general health education in English. It is not personal medical advice.

Diagnosis and treatment should be guided by a qualified clinician who knows your full history.

Seek urgent care for severe, sudden, or rapidly worsening symptoms.