What Is Vasculitis?
Vasculitis is a group of diseases characterised by inflammation of blood vessels (arteries, veins, or capillaries). When blood vessel walls become inflamed, they can narrow, block, or weaken — restricting blood flow and damaging organs. Vasculitis can affect virtually any organ system depending on which vessels are involved.
Vasculitis is broadly classified by vessel size: large-vessel, medium-vessel, and small-vessel vasculitis.
Types of Vasculitis Most Relevant to India
Takayasu Arteritis — India’s Most Common Large-Vessel Vasculitis
Takayasu Arteritis (TA) is a granulomatous inflammation of the aorta and its major branches. It disproportionately affects young Indian women aged 10–40, making India one of the highest-prevalence countries worldwide. The cause is unknown.
Symptoms: Arm or leg claudication (pain on exertion), difference in blood pressure between arms, absent pulses, hypertension, visual disturbances, dizziness, headaches, and sometimes fever and weight loss in the early inflammatory phase.
Diagnosis: CT angiography or MR angiography showing vessel wall thickening and stenosis of the aorta/branches. ESR and CRP are elevated in active disease. Angiography remains the gold standard for vascular mapping before intervention.
Treatment: High-dose prednisolone as initial therapy. Methotrexate, azathioprine, or mycophenolate for maintenance. TNF inhibitors (tocilizumab, infliximab) for refractory cases. Revascularisation (stenting or bypass surgery) when critical stenosis causes organ ischaemia — must only be done in remission.
ANCA-Associated Vasculitis (AAV)
ANCA (Anti-Neutrophil Cytoplasmic Antibody) vasculitis primarily affects small vessels. The main types are:
- Granulomatosis with Polyangiitis (GPA/Wegener’s) — affects upper airways (sinusitis, nosebleeds, nasal saddle deformity), lungs (haemoptysis, cavities), and kidneys (rapidly progressive nephritis)
- Microscopic Polyangiitis (MPA) — mainly kidneys and lungs
- Eosinophilic Granulomatosis with Polyangiitis (EGPA/Churg-Strauss) — asthma, eosinophilia, and systemic vasculitis
Key tests: ANCA (c-ANCA/PR3 in GPA; p-ANCA/MPO in MPA), kidney biopsy for nephritis confirmation.
Treatment: Cyclophosphamide + steroids for induction; azathioprine or MMF for maintenance. Rituximab is now preferred over cyclophosphamide in many centres for reduced toxicity.
Kawasaki Disease — Children’s Vasculitis
Kawasaki disease is an acute vasculitis of medium vessels predominantly affecting children under 5. It is the leading cause of acquired heart disease (coronary artery aneurysms) in children in India. Key features: fever >5 days, red cracked lips, strawberry tongue, red eyes without discharge, rash, and swollen hands/feet with desquamation. Treatment: IVIG + aspirin within 10 days of fever onset to prevent coronary complications.
IgA Vasculitis (Henoch-Schönlein Purpura)
The most common vasculitis in children. Causes palpable purpura on lower legs and buttocks, abdominal pain, joint pain, and kidney involvement. Usually self-limiting in children; can be more serious in adults.
Polyarteritis Nodosa (PAN)
Medium-vessel vasculitis causing renal artery involvement, mononeuritis multiplex (nerve damage), skin nodules, and abdominal pain. Associated with hepatitis B in some cases.
General Warning Signs of Vasculitis
- Unexplained fever for weeks with joint pain and weight loss
- Palpable purpura (raised red/purple spots) on skin
- Numbness, weakness, or pain in multiple nerve distributions
- Coughing blood (haemoptysis)
- Persistent sinusitis not responding to antibiotics
- Rapidly rising creatinine (kidney function worsening)
- Absent or markedly reduced pulses in limbs
Diagnosis of Vasculitis
Vasculitis diagnosis combines clinical features, blood tests (ANCA, complement, ESR/CRP), imaging (CT angiography, PET-CT), and tissue biopsy. The specific combination depends on the type of vasculitis suspected.
Treatment Principles
All vasculitis treatments involve immunosuppression to stop vessel inflammation. The standard approach is: induction therapy (high-dose immunosuppression to achieve remission) followed by maintenance therapy (lower-dose to prevent relapse). Regular monitoring for disease activity, treatment side effects, and organ damage is essential throughout.
Written by Dr. Keerthivardhan, Clinical Immunologist & Rheumatologist, NIMS Hospital Hyderabad.