What is ANCA Associated Vasculitis?
ANCA-Associated Vasculitis (AAV) is a group of three related small-vessel vasculitides that are defined by the presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCA): Granulomatosis with Polyangiitis (GPA, formerly Wegener's Granulomatosis), Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome). Together, these conditions cause devastating inflammation of the body's smallest blood vessels, most severely affecting the kidneys and lungs.
Without treatment, AAV — particularly GPA with rapidly progressive glomerulonephritis — can destroy both kidneys within weeks, leading to end-stage renal failure requiring dialysis. The speed of diagnosis and initiation of treatment is literally a life-and-kidney saving priority.
Saddle-nose deformity from cartilage destruction in GPA — a characteristic but late finding of aggressive upper airway granulomatous inflammation.
The Three Types of AAV
GPA (Granulomatosis with Polyangiitis)
GPA is characterised by granulomatous inflammation of the upper and lower respiratory tract PLUS small-vessel vasculitis causing glomerulonephritis. Classic features include: chronic sinusitis with bloody nasal discharge, nasal septal perforation leading to saddle-nose deformity, subglottic stenosis causing hoarseness, pulmonary nodules/cavities, and rapidly progressive glomerulonephritis. PR3-ANCA (c-ANCA) is the predominant antibody.
MPA (Microscopic Polyangiitis)
MPA causes small-vessel vasculitis affecting the kidneys (rapidly progressive glomerulonephritis) and lungs (pulmonary haemorrhage — a life-threatening emergency) without granulomatous inflammation. MPO-ANCA (p-ANCA) is the predominant antibody. The combination of pulmonary haemorrhage and renal failure is called "pulmonary-renal syndrome" and constitutes a rheumatological emergency.
EGPA (Eosinophilic GPA)
EGPA has three phases: allergic rhinitis and asthma → peripheral blood eosinophilia with eosinophilic tissue infiltration → systemic vasculitis. Mononeuritis multiplex (asymmetric nerve damage) and cardiac involvement (eosinophilic myocarditis — a major cause of mortality in EGPA) are critical manifestations.
HRCT chest showing bilateral ground-glass opacification from pulmonary haemorrhage — a life-threatening emergency in ANCA vasculitis.
🚨 Pulmonary-Renal Syndrome: A Rheumatological Emergency
The combination of coughing up blood (haemoptysis) AND kidney failure (haematuria, rising creatinine) in any patient demands immediate rheumatological assessment. This presentation can indicate ANCA vasculitis, Goodpasture's disease, or SLE. Without immediate treatment, both kidneys can be lost within days to weeks.
Dr. Prateek's Approach to ANCA Vasculitis
AAV is one of the true medical emergencies in Rheumatology. Speed of diagnosis, renal biopsy, and initiation of induction therapy can mean the difference between recovering renal function and permanent dialysis. Dr. Prateek Deo's specialist training at PGIMER included extensive experience with AAV management.
Urgent Diagnosis
Same-day ANCA testing, urgent kidney biopsy to classify histological severity, and immediate multidisciplinary team involvement for severe presentations.
Rituximab & Cyclophosphamide
We offer both approved induction regimens — Rituximab (preferred in GPA and for relapsing disease) and pulse IV Cyclophosphamide — with the choice guided by disease type, severity, and fertility considerations.
Plasma Exchange
For severe pulmonary haemorrhage or creatinine >500 µmol/L, we coordinate with nephrology for plasma exchange (plasmapheresis) to remove pathogenic ANCA antibodies rapidly.
Relapse Prevention
Long-term maintenance therapy with Rituximab or Azathioprine, with serial ANCA and urinalysis monitoring to detect early relapse before organ damage occurs.