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Granulomatosis with Polyangiitis (GPA)

Also known as: GPA, Wegener's Granulomatosis, WG, ANCA-associated vasculitis

Granulomatosis with Polyangiitis (GPA) is a rare autoimmune disease characterized by inflammation of blood vessels (vasculitis) in the nose, sinuses, ears, lungs, and kidneys. It is often associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs) in the blood. GPA can cause a range of symptoms and, if left untreated, can lead to organ damage and even death.

ICD-10: M31.3 Orphanet: 904 OMIM: 277900
👥 Estimated 3 cases per 100,000 people Prevalence
🔬 50 Active Trials

Imagine your body's defense system is attacking your blood vessels, especially in your nose, lungs, and kidneys. This causes swelling and damage, making it hard for those organs to work right. Doctors use strong medicines to calm down your defense system and stop it from attacking.

Signs & Symptoms

  • Nasal congestion and discharge
  • Sinus pain and infections
  • Cough
  • Shortness of breath
  • Hemoptysis (coughing up blood)
  • Joint pain
  • Skin rashes
  • Kidney inflammation (glomerulonephritis)
  • Fatigue
  • Fever
  • Weight loss
  • Eye inflammation (uveitis, scleritis)

Treatment Options

MEDICATION FDA Approved

Corticosteroids (e.g., Prednisone)

HIGHLY EFFECTIVE
MEDICATION FDA Approved

Immunosuppressants (e.g., Cyclophosphamide, Methotrexate, Azathioprine)

HIGHLY EFFECTIVE
MEDICATION FDA Approved

Rituximab

HIGHLY EFFECTIVE Approved 2011
THERAPY

Plasma exchange (Plasmapheresis)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Trimethoprim/sulfamethoxazole (Bactrim)

SUPPORTIVE

Diagnosis

  • Physical examination
  • Blood tests (ANCA, creatinine, ESR, CRP)
  • Urine tests
  • Chest X-ray or CT scan
  • Sinus CT scan
  • Kidney biopsy
  • Lung biopsy
  • Nasal biopsy

History

GPA was first described by Friedrich Wegener in 1936. It was originally known as Wegener's Granulomatosis. Due to Wegener's association with Nazi activities, there has been a movement to rename the disease to Granulomatosis with Polyangiitis (GPA).

Recent Breakthroughs

2011

Rituximab Approved for GPA

Rituximab was approved by the FDA for the treatment of GPA, providing an alternative to cyclophosphamide.

2017

Avacopan for ANCA-Associated Vasculitis

Avacopan, a selective C5a receptor inhibitor, has shown promise in clinical trials for the treatment of ANCA-associated vasculitis, including GPA, potentially reducing the need for high-dose corticosteroids.