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ANCA-associated vasculitis, large-vessel vasculitis, and organ damage assessment
Vasculitis encompasses a heterogeneous group of disorders characterised by inflammation of blood vessels that can affect any organ system and range from life-threatening systemic disease to isolated cutaneous involvement. At Dr. Shree Narayan's clinic, vasculitis classification follows the 2012 Chapel Hill nomenclature (large, medium, and small vessel), and assessment integrates ANCA serology (c-ANCA/PR3, p-ANCA/MPO), imaging (CT/MR angiography, PET-CT), and tissue biopsy where feasible. Treatment is stratified by disease severity using the BVAS (Birmingham Vasculitis Activity Score) and VDI (Vasculitis Damage Index), with induction immunosuppression transitioning to long-term maintenance to prevent relapse.
Kathmandu Neurology Clinic & Cognitive Center
Authored and reviewed by Dr. Shree Narayan Yadav (MBBS (KU), MD-Internal Medicine (NAMS), MSc Clinical Rheumatology (USW, UK), NMC 14227). Evidence-based, no fabricated outcomes.
Vasculitis encompasses a heterogeneous group of disorders characterised by inflammation of blood vessels that can affect any organ system and range from life-threatening systemic disease to isolated cutaneous involvement. At Dr. Shree Narayan's clinic, vasculitis classification follows the 2012 Chapel Hill nomenclature (large, medium, and small vessel), and assessment integrates ANCA serology (c-ANCA/PR3, p-ANCA/MPO), imaging (CT/MR angiography, PET-CT), and tissue biopsy where feasible. Treatment is stratified by disease severity using the BVAS (Birmingham Vasculitis Activity Score) and VDI (Vasculitis Damage Index), with induction immunosuppression transitioning to long-term maintenance to prevent relapse.
Reviewed by Dr. Shree Narayan Yadav, MBBS, MD, MSc — Consultant Physician & Joint and Autoimmune Disease Specialist (NMC 14227), Kathmandu Neurology Clinic. For evaluation, book an appointment.
The 2012 Chapel Hill Consensus Conference classifies vasculitis by vessel size. Large-vessel vasculitis includes giant cell arteritis (GCA) and Takayasu arteritis, affecting the aorta and its major branches. Medium-vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease, affecting medium-sized arteries. Small-vessel vasculitis includes ANCA-associated vasculitis (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], eosinophilic granulomatosis with polyangiitis [EGPA]) and immune complex vasculitis (IgA vasculitis, cryoglobulinaemic vasculitis, anti-GBM disease). Diagnosis requires integration of clinical features, serological markers, imaging, and where possible, histological confirmation.
ANCA-associated vasculitis treatment is guided by severity. Severe disease (renal, neurological, or pulmonary involvement) requires induction with rituximab or cyclophosphamide plus high-dose corticosteroids. The RAVE and RITUXVAS trials established rituximab as non-inferior to cyclophosphamide for remission induction and superior for relapsing disease. Maintenance therapy transitions to azathioprine or rituximab (MIRASTER trial: 50 mg every 6 months for 2 years). The ADVOCATE trial demonstrated avacopan (complement C5a receptor inhibitor) as a corticosteroid-sparing adjunct. Large-vessel vasculitis requires high-dose glucocorticoids with tocilizumab for GCA (GiACTA trial) or methotrexate for Takayasu.
Guidelines referenced:
Relapse occurs in 30-50% of ANCA-associated vasculitis patients, more commonly in PR3-positive disease. Monitoring includes serial ANCA titres (rising titres may predict relapse but are not alone sufficient for treatment escalation), renal function, urinalysis, inflammatory markers, and organ-specific assessment. The BVAS (Birmingham Vasculitis Activity Score) quantifies active disease, while the VDI (Vasculitis Damage Index) captures irreversible accumulated damage. Relapse management mirrors induction but with consideration of rituximab retreatment if the patient was previously on rituximab.
Stone JH et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis (RAVE trial). N Engl J MedJayne DR et al. Maintenance rituximab plus azathioprine for ANCA-associated vasculitis (MAINRITSAN). N Engl J MedHellmich B et al. EULAR/ERA recommendations for ANCA-associated vasculitis. Ann Rheum Dis 2023Content on this website is for general educational purposes only and does not constitute medical advice, diagnosis, or treatment. It is not a substitute for evaluation by a qualified healthcare professional. Always seek the advice of your physician or other qualified provider with any questions regarding a medical condition. Never disregard professional medical advice or delay seeking it because of something you have read on this website. In case of a medical emergency, contact emergency services immediately. No doctor–patient relationship is established by use of this site or by contacting the clinic through the site.