Autoimmune/Rheumatic

Granulomatosis with Polyangiitis (Wegener's)

Granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, is a rare disorder that causes inflammation of the blood vessels in the nose, sinuses, throat, lungs, and kidneys. It belongs to the group of vasculitis disorders. Untreated, GPA can be life-threatening due to organ damage.

9

Symptoms

3

Causes

4

Treatments

1

Prevention

Condition Overview

Understand key symptoms, causes, diagnosis options, and treatment pathways for Granulomatosis with Polyangiitis (Wegener's). This overview is intended for patient awareness and should be followed by specialist consultation.

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Symptoms

  • Runny nose, nosebleeds, sinus pain
  • Ear pain, hearing loss
  • Coughing (sometimes with blood)
  • Shortness of breath
  • Blood in urine (kidney involvement)
  • Joint pain
  • Eye inflammation
  • Skin rashes
  • Fever and fatigue

Causes

  • Autoimmune vasculitis
  • ANCA antibodies (PR3-ANCA/c-ANCA)
  • Exact trigger unknown

Diagnosis

  • Blood tests (ANCA — PR3/c-ANCA)
  • Urinalysis
  • Chest X-ray and CT
  • Biopsy of affected tissue
  • Kidney biopsy if nephritis suspected

Treatment

  • Cyclophosphamide + prednisone (induction for severe disease)
  • Rituximab (induction and maintenance)
  • Methotrexate or azathioprine (maintenance)
  • TMP-SMX for localized disease and infection prevention

Risk Factors

  • White ethnicity
  • Middle age
  • Possible genetic factors

Prevention

  • No known prevention

Prevalence

Rare; affects approximately 3 per 100,000 people in the United States.