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Health condition · Clinically reviewed

Granulomatosis with polyangiitis, ANCA vasculitis, rituximab and modern remission care.

Rare, serious and highly treatable. A specialist commissioned UK vasculitis service, modern induction with rituximab and avacopan, and lifelong monitoring keep most people in remission.

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Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced from guidance

    Checked against BSR, EULAR, KDIGO and NHS England specialist commissioning standards.

  • 03

    Current for 2026

    Reflects modern UK care including rituximab, avacopan (Tavneos) and specialist commissioned vasculitis services.

Key facts

GPA at a glance.

A rare but life-threatening ANCA-associated vasculitis - transformed over the last decade by rituximab, avacopan and specialist commissioned UK care.

  • What it is

    ANCA-associated small-to-medium vessel vasculitis with necrotising granulomatous inflammation of the airways, lungs and kidneys.

  • Also known as

    Wegener granulomatosis. Renamed granulomatosis with polyangiitis (GPA) in 2011.

  • Who it affects

    Rare - peak onset 40 to 60 years, affecting men and women roughly equally. A life-threatening condition without treatment.

  • Key antibody

    Typically c-ANCA with PR3 specificity (PR3-ANCA) - highly sensitive and specific in active disease.

  • Modern induction

    High-dose steroids with rituximab or cyclophosphamide, often alongside avacopan to spare steroids.

  • Where care happens

    Specialist commissioned UK vasculitis services (Cambridge, Newcastle, Birmingham, Barts and others) leading a rheumatology, nephrology and ENT MDT.

Why this guide matters

A rare disease that rewards early, specialist care.

The gap between untreated and modern outcomes is huge. Early ANCA testing, prompt referral and a specialist commissioned vasculitis MDT change the shape of the illness.

  • Test ANCA early

    c-ANCA with PR3 specificity is highly sensitive and specific. Testing early in unexplained sinus, lung or kidney disease shortens the road to diagnosis.

  • Rituximab and avacopan lead

    Modern induction pairs high-dose steroids with rituximab (see /treatments/rituximab-infusion-clinic/). Avacopan reduces steroid burden and long-term harm.

  • A commissioned MDT matters

    Specialist commissioned UK vasculitis services in Cambridge, Newcastle, Birmingham and Barts lead the rheumatology, nephrology and ENT MDT you need.

How the diagnosis is made

From first symptoms to a specialist plan.

The steps a UK team will normally follow, in order, based on BSR, EULAR and KDIGO guidance for ANCA-associated vasculitis.

  1. 01

    Assessing

    Multisystem clinical review

    A careful history and examination looking for airway, lung, kidney, skin, eye and nerve involvement.

  2. 02

    Assessing

    ANCA and PR3-ANCA testing

    c-ANCA with PR3 specificity is highly sensitive and specific for GPA and guides urgency of onward referral.

  3. 03

    Assessing

    Inflammatory markers and bloods

    CRP, ESR, FBC, U&Es and LFTs to gauge activity and end-organ involvement.

  4. 04

    Confirming

    Urinalysis and PCR

    Dipstick haematuria and proteinuria, urine protein-creatinine ratio or 24-hour collection to detect glomerulonephritis.

  5. 05

    Confirming

    Imaging - CT sinuses and chest

    Looking for sinus disease, cavitating pulmonary nodules and pulmonary haemorrhage.

  6. 06

    Confirming

    Tissue biopsy

    Renal, lung or nasal biopsy confirms necrotising granulomatous inflammation and pauci-immune crescentic glomerulonephritis.

  7. 07

    Preparing

    Specialist vasculitis MDT

    BVAS and VDI scoring, and referral to a specialist commissioned UK vasculitis service for induction and maintenance planning.

Typical timeline: from suspicion to specialist MDT plan in days when the presentation is severe.

Symptoms

What GPA can look like.

A truly multisystem disease. The pattern varies from patient to patient, but airway, lung and kidney disease dominate the picture.

  • Upper airway - saddle nose and crusting

    Chronic sinusitis, epistaxis, nasal crusting, otitis media, oral ulceration and, in advanced disease, a saddle-nose deformity or subglottic stenosis.

  • Lower respiratory - haemoptysis

    Coughing up blood, breathlessness, pulmonary haemorrhage and cavitating lung nodules on imaging.

  • Kidneys - rapidly progressive GN

    Haematuria, proteinuria and rising creatinine from pauci-immune crescentic glomerulonephritis. See /conditions/glomerulonephritis/.

  • Skin - purpura and ulcers

    Palpable purpura, necrotising vasculitic ulcers and nodules, often on the lower limbs.

  • Eyes - scleritis and orbital disease

    Painful red eye from scleritis, episcleritis or an orbital pseudotumour that can threaten vision.

  • Nerves - mononeuritis multiplex

    Patchy peripheral neuropathy affecting individual nerves, and less commonly central nervous system involvement.

  • Systemic - fever, weight loss, malaise

    Constitutional symptoms including fatigue, night sweats and unintentional weight loss - often the earliest clue.

  • Red flag - pulmonary-renal syndrome

    Combined haemoptysis and rising creatinine is a medical emergency needing immediate specialist admission.

Treatment

How GPA is treated in the UK.

High-dose steroids with rituximab or cyclophosphamide for induction, avacopan to spare steroids, then maintenance therapy - all through a specialist commissioned vasculitis service.

  • High-dose corticosteroids

    Intravenous methylprednisolone followed by oral prednisolone as the initial anti-inflammatory backbone during induction.

  • Rituximab induction

    Anti-CD20 monoclonal antibody, given as an infusion course - the modern first-line induction agent for most patients. See /treatments/rituximab-infusion-clinic/.

  • Cyclophosphamide induction

    A traditional alternative to rituximab, still used in selected severe or resistant cases under specialist care.

  • Avacopan (Tavneos)

    Oral complement C5a receptor inhibitor, NICE-approved in 2022 to reduce steroid burden in active ANCA-associated vasculitis. See /treatments/avacopan-anca-vasculitis-clinic/.

  • Maintenance therapy

    Rituximab, azathioprine, methotrexate or mycophenolate to keep the disease in remission once induction is complete.

  • Plasma exchange

    Reserved for severe pulmonary haemorrhage or dialysis-dependent renal failure at presentation, under specialist commissioned care.

  • Supportive care

    PJP prophylaxis with co-trimoxazole, bone protection against steroid-induced osteoporosis and a planned vaccination schedule.

  • Surgical and organ support

    Dilatation for subglottic stenosis, plastic surgical nasal reconstruction, and dialysis or transplantation for end-stage renal failure.

What this guide is based on

The sources behind every claim on this page.

UK national guidance and international specialist society standards, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your rheumatology, nephrology or ENT team knows your case and can tell you which parts apply to you. If in doubt, contact your specialist nurse or GP.

  • British Society for Rheumatology (BSR). Guideline for the management of ANCA-associated vasculitis.

  • EULAR. Recommendations for the management of ANCA-associated vasculitis.

  • KDIGO. Clinical practice guideline for the management of glomerular diseases (ANCA vasculitis).

  • NICE. Avacopan for treating severe active granulomatosis with polyangiitis or microscopic polyangiitis (TA825).

  • NHS England. Specialised commissioning service specification for adult vasculitis services.

Red flags

When GPA needs urgent attention.

These are the situations where waiting is not safe - contact your specialist team, GP or emergency services the same day.

  • Pulmonary haemorrhage

    Frank haemoptysis with breathlessness or falling oxygen saturations is a medical emergency needing immediate hospital admission.

  • Rapidly rising creatinine

    A sudden climb in creatinine with haematuria and proteinuria suggests rapidly progressive glomerulonephritis and needs same-day specialist input.

  • Vision-threatening eye disease

    Sudden painful red eye, reduced vision or proptosis from scleritis or orbital pseudotumour needs urgent ophthalmology review.

  • Subglottic stenosis

    Stridor, hoarseness or worsening breathlessness from airway narrowing may need urgent ENT assessment and dilatation.

  • New neurological deficit

    Sudden weakness, foot drop or sensory loss can indicate mononeuritis multiplex or central nervous system vasculitis.

  • Sepsis on immunosuppression

    Fever, rigors or a rising CRP in a patient on rituximab, cyclophosphamide or high-dose steroids needs urgent assessment.

  • PJP pneumonia

    Progressive breathlessness and dry cough in an immunosuppressed patient - a reminder that co-trimoxazole prophylaxis matters.

  • Relapse after remission

    Return of nasal crusting, haematuria, rising ANCA or new pulmonary nodules warrants prompt specialist review.

  • Steroid toxicity

    New diabetes, avascular necrosis, severe osteoporosis or mood change on long-course steroids needs urgent rebalancing of therapy.

Living with it

A serious condition, manageable with structure.

Long-term GPA care is a partnership - shared between you, your commissioned specialist team and your GP. Four habits make the biggest difference.

A quiet reminder

Rare does not mean alone.

Vasculitis UK and the clinical nurse specialists at your commissioned centre are there between clinic appointments - use them.

  1. 01 MDT

    Stay linked to your specialist team

    Rheumatology, nephrology and ENT clinicians share the load - keep every appointment and know who to call between visits.

  2. 02 Monitoring

    Regular bloods and urine checks

    ANCA levels, kidney function and urinalysis guide relapse detection. Small changes matter - do not skip surveillance.

  3. 03 Prevention

    Vaccines, bones and PJP cover

    Annual flu, COVID and pneumococcal jabs, plus bone protection and co-trimoxazole prophylaxis, protect against the main treatment risks.

  4. 04 Support

    Use Vasculitis UK and the MDT

    Peer support from Vasculitis UK, and clinical nurse specialists at your commissioned centre, help make a rare condition feel less isolating.

Frequently asked

Everything we get asked about GPA.

Quick answers on ANCA testing, rituximab, avacopan, remission and long-term monitoring.

  • What is granulomatosis with polyangiitis?

    GPA (formerly Wegener granulomatosis) is a rare autoimmune small-to-medium vessel vasculitis. It causes necrotising granulomatous inflammation of blood vessels, most often affecting the upper airway, lungs and kidneys, and is strongly associated with c-ANCA and PR3-ANCA antibodies.

  • How is GPA diagnosed?

    Diagnosis rests on multisystem clinical features, positive ANCA (usually c-ANCA with PR3 specificity), inflammatory markers, imaging of the sinuses and chest, urinalysis with a protein-creatinine ratio, and tissue biopsy - typically of the kidney, lung or nose - showing necrotising granulomatous inflammation and pauci-immune crescentic glomerulonephritis.

  • What is the modern first-line treatment?

    For most patients, induction is high-dose corticosteroids alongside rituximab, an anti-CD20 monoclonal antibody. Cyclophosphamide is an alternative in selected cases. Avacopan (Tavneos) is now used in eligible patients to reduce the steroid burden. Care is coordinated by a specialist commissioned UK vasculitis service.

  • What is avacopan and why does it matter?

    Avacopan (Tavneos) is an oral complement C5a receptor inhibitor. NICE approved it in 2022 for severe active GPA and microscopic polyangiitis. It allows the same or better rates of sustained remission while reducing the cumulative steroid dose, which limits long-term steroid harms.

  • Is GPA curable?

    GPA is not curable but is highly treatable. Modern induction and maintenance regimens put most patients into sustained remission. Relapses do occur, so long-term specialist follow-up, regular monitoring of ANCA and organ function, and prompt reassessment of any new symptoms are essential.

  • What are the main long-term risks?

    The main risks are relapse, chronic kidney disease progressing to dialysis or transplantation, infections on immunosuppression (including PJP pneumonia), steroid-related complications such as osteoporosis and diabetes, subglottic stenosis, hearing loss and permanent nasal or facial changes such as a saddle-nose deformity.

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