Vasculitis

Overview

Vasculitis is an inflammatory condition that affects blood vessels, causing them to become inflamed, weakened, narrowed, or scarred. This inflammation can affect arteries, veins, and capillaries of various sizes throughout the body. When blood vessels are inflamed, blood flow to organs and tissues is reduced, potentially causing serious damage.

The severity and impact of vasculitis depend on which blood vessels are affected and how extensively. Some forms of vasculitis are acute and resolve on their own, while others are chronic and require long-term management. The condition can occur as a primary disorder or secondary to other diseases, infections, or medications.

Understanding vasculitis is crucial because early diagnosis and treatment can prevent serious complications. While some types affect only the skin and resolve without treatment, others can damage vital organs including the kidneys, lungs, heart, and brain. Modern treatments have significantly improved outcomes for people with vasculitis, making it a manageable condition for many patients.

Symptoms

Vasculitis symptoms vary widely depending on which blood vessels and organs are affected:

General Symptoms

• Fever
• Fatigue and weakness
• Weight loss
• Muscle and joint pain
• Loss of appetite
• Night sweats

Specific Symptoms by Location

• Headache - Especially with temporal arteritis
• Skin rash - Purple spots (purpura), ulcers, or nodules
• Skin lesions - Painful bumps or open sores
• Leg pain - From reduced blood flow
• Foot or toe swelling - Due to inflammation
• Leg stiffness or tightness - From vascular involvement
• Lymphedema - Swelling from lymphatic involvement
• Dizziness - From blood pressure changes or brain involvement

Organ-Specific Symptoms

• Eyes: Blurred vision, sudden blindness, eye pain
• Lungs: Shortness of breath, cough, chest pain, blood in sputum
• Kidneys: Blood in urine, decreased kidney function
• Nerves: Numbness, tingling, weakness
• GI tract: Abdominal pain, blood in stool
• Heart: Chest pain, heart attack symptoms

Types of Vasculitis

Vasculitis is classified based on the size of affected blood vessels:

Large Vessel Vasculitis

• Giant Cell Arteritis (GCA/Temporal Arteritis):
  • Most common form in adults over 50
  • Affects arteries in head, especially temporal arteries
  • Can cause blindness if untreated
• Takayasu Arteritis:
  • Affects aorta and major branches
  • More common in young women
  • Can cause arm claudication and blood pressure differences

Medium Vessel Vasculitis

• Polyarteritis Nodosa (PAN):
  • Affects medium-sized arteries
  • Can damage kidneys, intestines, nerves
  • Associated with hepatitis B in some cases
• Kawasaki Disease:
  • Primarily affects children under 5
  • Can damage coronary arteries
  • Leading cause of acquired heart disease in children

Small Vessel Vasculitis

• Granulomatosis with Polyangiitis (GPA):
  • Affects nose, sinuses, lungs, kidneys
  • Can cause saddle-nose deformity
  • ANCA-positive in most cases
• Microscopic Polyangiitis:
  • Affects small vessels in kidneys and lungs
  • Can cause rapidly progressive glomerulonephritis
• IgA Vasculitis (Henoch-Schönlein Purpura):
  • Most common vasculitis in children
  • Characteristic purpuric rash on legs
  • Usually self-limiting

Variable Vessel Vasculitis

• Behçet's Disease:
  • Causes oral and genital ulcers
  • Eye inflammation common
  • More prevalent along ancient Silk Road
• Cogan's Syndrome:
  • Affects eyes and ears
  • Can cause hearing loss and vision problems

Causes

The exact cause of most vasculitis types is unknown, but several factors can trigger the condition:

Autoimmune Response

• Immune system mistakenly attacks blood vessels
• Production of autoantibodies (like ANCA)
• Formation of immune complexes that deposit in vessel walls
• T-cell mediated inflammation

Infections

• Hepatitis B and C: Associated with PAN and cryoglobulinemic vasculitis
• HIV: Can trigger various vasculitis types
• Bacterial infections: Streptococcus, staphylococcus
• Other viruses: Parvovirus B19, cytomegalovirus

Medications

• Antibiotics (especially sulfonamides)
• Hydralazine
• Allopurinol
• Propylthiouracil
• Tumor necrosis factor inhibitors
• Cocaine (contaminated with levamisole)

Other Triggers

• Cancer: Paraneoplastic vasculitis
• Other autoimmune diseases: Lupus, rheumatoid arthritis
• Genetic factors: HLA associations in some types
• Environmental factors: Silica exposure, smoking

Risk Factors

Various factors can increase the risk of developing vasculitis:

Demographic Factors

• Age:
  • GCA: Over 50 years
  • Kawasaki: Under 5 years
  • Takayasu: 15-40 years
• Gender:
  • Takayasu: More common in women
  • PAN: More common in men
• Ethnicity:
  • Takayasu: Asian populations
  • Behçet's: Mediterranean, Middle Eastern, Asian
  • GCA: Northern European descent

Medical Conditions

• Hepatitis B or C infection
• Autoimmune diseases
• Cancer (especially blood cancers)
• Chronic infections

Genetic Factors

• Family history of vasculitis
• Specific HLA types
• Genetic polymorphisms in immune genes

Environmental Factors

• Smoking
• Occupational exposures (silica, pesticides)
• Geographic location
• Seasonal variations (some types)

Diagnosis

Diagnosing vasculitis requires a combination of clinical assessment and testing:

Medical History and Physical Exam

• Detailed symptom history
• Medication review
• Family history
• Complete physical examination
• Blood pressure in multiple limbs

Blood Tests

• Inflammatory markers:
  • ESR (erythrocyte sedimentation rate)
  • CRP (C-reactive protein)
• Autoantibodies:
  • ANCA (c-ANCA, p-ANCA)
  • ANA (antinuclear antibodies)
  • Anti-GBM antibodies
  • Cryoglobulins
• Other tests:
  • Complete blood count
  • Kidney function tests
  • Liver function tests
  • Complement levels

Imaging Studies

• Angiography: Gold standard for large vessel vasculitis
• CT angiography: Non-invasive vessel imaging
• MRI/MRA: Detailed vessel and organ imaging
• PET scan: Shows inflammation in vessel walls
• Ultrasound: For temporal arteries and large vessels
• Chest X-ray: Lung involvement

Tissue Biopsy

• Temporal artery biopsy: For GCA diagnosis
• Skin biopsy: For cutaneous vasculitis
• Kidney biopsy: If kidney involvement
• Nerve/muscle biopsy: For PAN
• Lung biopsy: For pulmonary vasculitis

Other Tests

• Urinalysis (protein, blood, casts)
• Pulmonary function tests
• Nerve conduction studies
• Eye examination

Treatment

Treatment aims to reduce inflammation, prevent organ damage, and achieve remission:

Corticosteroids

• High-dose for severe disease:
  • Prednisone 1mg/kg/day initially
  • IV methylprednisolone for critical cases
  • Gradual tapering over months
• Side effect management:
  • Bone protection (calcium, vitamin D)
  • Gastric protection
  • Blood sugar monitoring
  • Infection prevention

Immunosuppressive Medications

• Cyclophosphamide: For severe organ-threatening disease
• Methotrexate: Steroid-sparing agent
• Azathioprine: Maintenance therapy
• Mycophenolate mofetil: Alternative for maintenance
• Leflunomide: For specific types

Biologic Therapies

• Rituximab: For ANCA-associated vasculitis
• Tocilizumab: For giant cell arteritis
• TNF inhibitors: For Behçet's, Takayasu
• Mepolizumab: For eosinophilic GPA

Other Treatments

• Plasma exchange: For severe kidney or lung disease
• Antiplatelet therapy: Low-dose aspirin
• Anticoagulation: For thrombosis risk
• Antibiotics: If infection-related
• Surgery: For complications (aneurysms, stenosis)

Supportive Care

• Blood pressure management
• Pain control
• Physical therapy
• Nutritional support
• Psychological support

Long-term Management

Vasculitis often requires ongoing monitoring and management:

Disease Monitoring

• Regular clinical assessments
• Inflammatory marker monitoring
• Organ function tests
• Imaging surveillance
• Drug level monitoring

Medication Management

• Gradual steroid tapering
• Maintenance immunosuppression
• Side effect monitoring
• Vaccination updates (before immunosuppression)
• Prophylactic medications

Lifestyle Modifications

• Smoking cessation
• Regular exercise as tolerated
• Heart-healthy diet
• Stress management
• Sun protection (with certain medications)

Preventing Complications

• Infection prevention strategies
• Cardiovascular risk reduction
• Osteoporosis prevention
• Cancer screening
• Eye exams (for at-risk patients)

Complications

Untreated or severe vasculitis can lead to serious complications:

Organ Damage

• Kidney failure: From glomerulonephritis
• Lung damage: Pulmonary hemorrhage, fibrosis
• Heart problems: Heart attack, heart failure
• Stroke: From cerebral vasculitis
• Blindness: Especially with GCA
• Peripheral neuropathy: Nerve damage

Vascular Complications

• Aneurysms (vessel bulging)
• Stenosis (vessel narrowing)
• Thrombosis (blood clots)
• Hemorrhage (bleeding)
• Tissue ischemia and necrosis

Treatment Complications

• Steroid side effects
• Increased infection risk
• Secondary cancers
• Infertility from cyclophosphamide
• Drug toxicity

Quality of Life Impact

• Chronic pain
• Fatigue
• Depression and anxiety
• Work disability
• Social isolation

When to See a Doctor

Seek medical attention for symptoms suggesting vasculitis:

Emergency Symptoms

• Sudden vision loss or changes
• Severe headache with jaw pain
• Chest pain or difficulty breathing
• Coughing up blood
• Severe abdominal pain
• Signs of stroke
• High fever with rash

Urgent Evaluation Needed

• Unexplained fever and weight loss
• New skin rash with systemic symptoms
• Persistent headaches
• Multiple organ symptoms
• Blood in urine or stool
• Numbness or weakness

For Diagnosed Patients

• Worsening symptoms despite treatment
• New symptoms developing
• Medication side effects
• Signs of infection
• Pregnancy planning

Regular Follow-up

• As scheduled by rheumatologist
• Laboratory monitoring
• Medication adjustments
• Annual comprehensive evaluations