Granulomatosis with Polyangiitis (Wegener’s)

What is Granulomatosis with Polyangiitis?

Granulomatosis with Polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare form of vasculitis mainly involving small and medium-sized blood vessels. The disease commonly affects the nose, sinuses, throat, lungs, ears, eyes, kidneys, skin, joints, and nerves. Occasionally GPA can also affect the heart, intestines, brain, or other organs.

Not everyone with GPA is affected in the same way. Some patients have mild disease, while others may have severe damage to these organs that can be life-threatening.

Who gets Granulomatosis with Polyangiitis?

GPA occurs in both men and women and can affect children and adults. Although Caucasians are more likely to get this type of vasculitis, people from all over the world can get GPA.

What causes Granulomatosis with Polyangiitis?

GPA is thought to be an auto-immune disease, the cause of which is unknown.

How is Granulomatosis with Polyangiitis diagnosed?

The diagnosis of GPA is made by combining clinical features with laboratory tests (including blood tests for ANCA antibodies) and tissue biopsies.

What is the treatment for Granulomatosis with Polyangiitis?

Treatment of GPA usually includes a combination of prednisone and an immunosuppressive drug such as cyclophosphamide, rituximab, methotrexate, or azathioprine. If diagnosed promptly, treatment can produce remission and prevent organ damage. GPA is usually, but not always, a chronic disease. Some patients have only one episode, while others have relapses separated by periods of remission, and others require long-term treatment to control daily symptoms.

*Note: This content has been adapted from the VCRC website