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Granulomatosis with Polyangiitis (GPA)

Formerly Wegener granulomatosis, an ANCA-associated vasculitis affecting the upper respiratory tract, lungs, and kidneys.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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

What is Granulomatosis with Polyangiitis (GPA)?

Granulomatosis with polyangiitis (GPA) is an ANCA-associated necrotizing granulomatous vasculitis with a predilection for the upper and lower respiratory tract and kidneys. PR3-ANCA (c-ANCA) positivity is typical.

Its classic triad includes upper airway involvement (sinusitis, nasal ulcers, saddle nose), lower airway involvement (lung nodules, cavitations, hemoptysis), and glomerulonephritis. Skin, eye, and nervous system can also be involved.

Early diagnosis is life-saving. Steroid combined with cyclophosphamide or rituximab is used for remission induction. Maintenance therapy is long-term.

Symptoms

Chronic sinusitis and nasal congestion
Saddle nose deformity
Hemoptysis
Chest pain
Hematuria and proteinuria
Fever and weight loss
Skin rash

Risk Factors

Genetic predisposition (HLA-DP)
Silica exposure
Infectious triggers
Middle age
Smoking
Coexisting autoimmune diagnoses
History of chronic sinusitis

When to See a Doctor?

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

  • Sinusitis not responding to treatment
  • Hemoptysis and renal findings
  • Saddle nose deformity
  • Signs of multi-organ involvement

Treatment Methods

01
Remission induction: high-dose steroid + cyclophosphamide or rituximab
02
Maintenance: azathioprine, methotrexate, or rituximab
03
Plasmapheresis (in severe cases)
04
PJP prophylaxis
05
Vaccination
06
Multidisciplinary follow-up

Which Department to Visit?

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

Learn About Göğüs Hastalıkları Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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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.