Granulomatosis with Polyangiitis

What is Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, is an autoimmune disorder that causes inflammation, swelling, and irritation in blood vessels and other tissues.

This inflammation reduces or stops the flow of blood to organs in the body. The condition mostly affects the respiratory system, including the sinuses, nose, windpipe, and lungs. It can also affect the kidneys and other organs in the body.

Ongoing GPA can have serious complications.

Most people with GPA first have vague symptoms that may include:

Tiredness or exhaustion
Joint pain
Upper respiratory symptoms that don’t respond to treatments for allergies or colds.
Eye infections, redness, burning, or pain
Weakness
Skin sores or ulcers
Hoarseness
Fever
Night sweats

UR Medicine's Treatments for Granulomatosis with Polyangiitis

Since this disease affects many parts of the body, more than one specialist may be involved in diagnosing and managing your illness. Specialists may include a pulmonologist (lungs), nephrologist (kidneys) and rheumatologist (autoimmune diseases).

To diagnose GPA, medical professionals will first take a detailed medical history and carry out a physical examination. They will then order various diagnostic tests, such as blood work, urine studies, and imaging tests. In some cases, a kidney biopsy may be warranted. While these tests can help to rule out other potential causes of your symptoms, a biopsy—which involves examining a tissue sample—is the only way to definitely diagnose GPA.

Common medicines used in treatment include:

Corticosteroids. This helps reduce inflammation.
Rituximab. This is an antibody against certain immune cells (B-cells) that can the autoimmune problem in GPA.
Cyclophosphamide. This is an immunosuppressive medication.

GPA may come back even after successful treatment. Continue to follow up with your healthcare provider, even when you are in remission.

What Sets Us Apart?

U.S. News & World Reports has identified us as one of the top adult nephrology (kidney) care centers in the country.

Our collaborative approach brings a wide range of expertise and diverse perspectives to patient care, with specialists in cardiology, endocrinology, rheumatology, transplant surgery, and urology working together to develop the best treatments for each patient.

Because we’re an academic medical center, our physicians also lead important research studies, with significant grants from the National Institutes of Health and industry-sponsored clinical trials.