Granulomatosis with Polyangiitis (Wegener)

ANCA-associated vasculitis affecting the upper airway, lungs and kidneys.

Written by: Saygı Hospital Health Guide Editorial Board

Last updated: June 9, 2026

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Granulomatosis with Polyangiitis (Wegener)?

Granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, is an ANCA-associated systemic vasculitis of small- and medium-sized vessels with necrotising granulomatous inflammation. The classic triad involves the upper airway, lungs and kidneys.

c-ANCA (anti-PR3) is positive in 80-90% of cases and is essential for diagnosis. Disease can be limited (upper airway only) or generalised (multi-organ involvement).

Untreated GPA carries very high mortality, but with modern immunosuppressive regimens remission rates exceed 70-90%. Relapse risk is high and long-term follow-up is required.

Symptoms

Chronic sinusitis and bloody nasal discharge

Saddle-nose deformity from nasal-bridge collapse

Pulmonary nodules and cavitations

Haemoptysis

Glomerulonephritis with rapidly progressive renal failure

Eye involvement (scleritis, orbital pseudotumour)

Cutaneous vasculitis and peripheral neuropathy

Risk Factors

White ethnicity (more common)

Age 40-65

Northern European ancestry

Genetic predisposition (HLA-DPB1)

Environmental triggers (infections, silica exposure)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

Chronic sinusitis with bloody discharge that does not respond to treatment
Haemoptysis with pulmonary infiltrates
Rapidly worsening renal function
Detection of c-ANCA positivity

Treatment Methods

Induction: high-dose corticosteroid plus cyclophosphamide or rituximab

Maintenance: azathioprine, methotrexate or rituximab

Trimethoprim-sulfamethoxazole (to suppress nasal carriage and upper-airway relapse)

Plasma exchange (in severe renal involvement or pulmonary haemorrhage)

Avacopan (complement C5a-receptor inhibitor — newer option)

Regular ANCA titre and organ-function monitoring

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dahiliye (İç Hastalıkları) Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.