Vasculitis

Symptoms

•  Systemic Symptoms: These include fever, fatigue, weight loss, and malaise, which are common in most forms of vasculitis due to widespread inflammation.

• Skin Changes: Red or purple spots (purpura), rashes, ulcers, or sores, especially on the legs, are common in small-vessel vasculitis.

• Organ-Specific Symptoms:
  • Kidney Involvement: Hematuria (blood in the urine), proteinuria, and signs of kidney failure in conditions like granulomatosis with polyangiitis (GPA) or Goodpasture’s syndrome.
  • Lung Symptoms: Cough, hemoptysis (coughing up blood), shortness of breath, or chest pain, seen in granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA).
  • Nervous System: Neurological symptoms such as weakness, numbness, or seizures may occur with large or medium vessel vasculitis.
  • Joint Pain: Arthritis or arthralgia (joint pain) is common in various types of vasculitis, especially in rheumatoid vasculitis.
  • Gastrointestinal Symptoms: Abdominal pain, bleeding, or perforation can occur if vasculitis affects the gastrointestinal tract.
• Eye Symptoms: Uveitis or conjunctivitis in conditions like Behçet's disease or takayasu arteritis

How is Diagnosis done ?

• Medical History and Physical Examination: A detailed history of symptoms, including systemic signs like fever and fatigue, along with an examination for signs of organ involvement (skin changes, joint inflammation, etc.).
• Blood Tests:
  • Inflammatory Markers: C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are typically elevated in active vasculitis.
  • ANCA (Anti-Neutrophil Cytoplasmic Antibodies): Tests for c-ANCA and p-ANCA are commonly used to diagnose granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (EGPA).
  • Complement Levels: Low C3 and C4 levels may suggest systemic vasculitis, especially in lupus vasculitis.
  • Kidney Function Tests: Blood urea nitrogen (BUN), creatinine, and urine tests for hematuria and proteinuria to assess renal involvement.
• Biopsy:
  • Tissue Biopsy: A biopsy of affected tissue (skin, kidney, lung, etc.) can confirm the diagnosis by showing signs of vasculitis (inflammation and blood vessel damage).
  • Vessel Biopsy: In cases of large vessel vasculitis like Takayasu arteritis or giant cell arteritis, a biopsy of the affected artery (such as temporal artery biopsy) is done.
• Imaging:
  • Angiography: Imaging techniques like CT angiography or MRI angiography are used to visualize blood vessels, assess for stenosis, aneurysms, or other vascular abnormalities.
  • Ultrasound: Doppler ultrasound may be used to detect blood flow abnormalities in affected arteries or veins.
• Pulmonary Function Tests: These are especially useful when lung involvement is suspected (e.g., GPA or EGPA).
• Urinalysis: Used to detect kidney damage, hematuria, or proteinuria, which can indicate glomerulonephritis in vasculitis.
  

Why is Early Diagnosis Important?

•  Preventing Organ Damage: Early diagnosis can prevent irreversible damage to critical organs such as the kidneys, lungs, and heart.
• Tailored Treatment: Early identification allows for the initiation of appropriate treatment, such as immunosuppressive therapy or biologics, to control inflammation and prevent flare-ups.
• Preventing Severe Complications: Vasculitis can lead to life-threatening complications like kidney failure, heart attack, stroke, or sepsis if left untreated. Prompt treatment can reduce these risks.
• Improving Prognosis: Many forms of vasculitis have a better long-term prognosis if diagnosed and treated early, leading to remission or better management of symptoms.
• Preventing Relapses: Monitoring and early treatment can help minimize relapses and allow for better control of the disease.

Treatment

• Immunosuppressive Medications:
• • Corticosteroids: Prednisone is often used to reduce inflammation, but long-term use requires careful monitoring.
  • Cyclophosphamide: A potent immunosuppressant used for severe or life-threatening forms of vasculitis, often in combination with corticosteroids.
  • Methotrexate and Azathioprine: These are often used in less severe cases or for maintenance therapy.
  • Rituximab: A monoclonal antibody targeting B-cells, used for refractory or severe cases of vasculitis, particularly ANCA-associated vasculitis.
• Biologic Therapy:
  • Tocilizumab: An interleukin-6 (IL-6) inhibitor, used in giant cell arteritis and other forms of vasculitis.
  • Infliximab and Adalimumab: These TNF inhibitors are used for treating certain types of vasculitis, especially Behçet’s disease.
• Plasmapheresis: In severe cases, especially in Goodpasture syndrome or ANCA-associated vasculitis, plasmapheresis (plasma exchange) may be used to remove antibodies from the bloodstream.
• Supportive Care:
  • Renal Replacement Therapy: In cases of kidney failure, dialysis may be necessary.
  • Pain and Symptom Management: NSAIDs, pain relievers, or physical therapy may be required for managing joint pain and other systemic symptoms.
• Surgical Interventions: In rare cases, surgery may be needed to treat complications, such as vascular repair or removal of damaged tissue (e.g., lung or kidney surgery).

Prognosis

• Variable Prognosis: The outlook for vasculitis depends largely on the type of vasculitis, the organs involved, and how early the disease is diagnosed and treated.
• Short-Term: If treated promptly with immunosuppressive therapy, many patients achieve remission or a significant reduction in symptoms, though relapses are possible.
• Organ Damage: Chronic vasculitis can cause lasting damage to organs such as the kidneys, heart, or lungs, which may lead to long-term complications or disability.
• Life Expectancy: While some types of vasculitis (e.g., giant cell arteritis or Takayasu arteritis) have a good long-term prognosis with proper treatment, others (e.g., granulomatosis with polyangiitis (GPA) or microscopic polyangiitis) may have a higher risk of serious complications or death due to organ failure or infections.
• Relapses: Many forms of vasculitis can relapse, requiring continuous monitoring and possibly maintenance therapy to keep the disease in remission.
• Mortality: The mortality rate for vasculitis has decreased significantly with improved treatments, but it remains higher for patients with ANCA-associated vasculitis or systemic vasculitis that causes severe organ damage.

Scoring

Several scoring systems and tools are used to assess the severity and monitor the progression of vasculitis:

• The Birmingham Vasculitis Activity Score (BVAS): A widely used tool to assess disease activity in ANCA-associated vasculitis and other forms of systemic vasculitis. It measures clinical signs and symptoms of vasculitis across multiple organ systems, such as skin, kidneys, lungs, and nerves.
• The VASculitis Damage Index (VDI): This tool evaluates permanent damage caused by vasculitis, considering both clinical findings and long-term effects of treatment. It helps in determining how much damage has occurred due to the disease and its treatment.
• The Modified Disease Activity Score for Giant Cell Arteritis (GCA): This score is used specifically for giant cell arteritis to measure disease activity based on symptoms like fever, visual disturbances, and blood vessel inflammation.
• The Vasculitis Activity Index (VAI): A comprehensive scoring system that assesses the activity of vasculitis in a variety of forms, including both large and small vessel involvement.
• The Patient-Reported Outcome Measures (PROMs): These tools, such as the VAS pain score and other questionnaires, help assess the patient’s experience with disease symptoms, such as pain, fatigue, and quality of life.

These scoring systems guide clinicians in monitoring disease progression, evaluating treatment effectiveness, and predicting potential complications.