Granulomatosis with Polyangiitis (GPA)

What is GPA?

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disorder that causes inflammation of blood vessels (vasculitis), affecting multiple organs in the body.

Key Features

• Blood vessel inflammation: Primarily affects small and medium-sized blood vessels
• Organ involvement: Commonly affects respiratory tract, kidneys, and sinuses
• Autoimmune nature: Body's immune system mistakenly attacks its own tissues

Common Symptoms

Upper Respiratory

• Persistent nasal congestion
• Sinus pain and infections
• Nosebleeds
• Hearing problems

Lungs

• Cough, sometimes with blood
• Shortness of breath
• Chest pain

Kidneys

• Blood in urine
• Decreased urine output
• Elevated blood pressure

Diagnosis

• Blood tests: ANCA (Anti-neutrophil cytoplasmic antibodies) testing
• Imaging: Chest X-rays, CT scans
• Biopsy: Tissue sampling from affected organs

Treatment Options

Initial Treatment

1. High-dose corticosteroids
2. Immunosuppressive medications
3. Rituximab or cyclophosphamide

Maintenance Therapy

• Lower-dose medications
• Regular monitoring
• Prevention of infections

Living with GPA

Self-Management

• Regular medical check-ups
• Medication compliance
• Infection prevention
• Healthy lifestyle maintenance

Warning Signs

• New or worsening symptoms
• Fever
• Unusual fatigue
• Changes in urination

Prognosis

With proper treatment, many patients achieve remission. Long-term monitoring is essential to prevent and manage flares.