Granulomatosis with Polyangiitis (GPA)

A rare form of vasculitis, granulomatosis with polyangiitis (GPA) causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. GPA is an autoimmune disease, which means your immune system mistakenly attacks your blood vessels.

Formerly known as Wegener’s granulomatosis, GPA produces granulomas, or nodules of inflamed tissue, around your blood vessels. Sometimes these granulomas develop in your respiratory tract or kidneys, which can have serious consequences. Serious complications, including kidney failure, usually result from a lack of treatment.

Symptoms of GPA

GPA is most common in people ages 40 to 65. It is not known what causes GPA. It may be triggered by an infection. There is no evidence that GPA is inherited, but some people may have a genetic predisposition.

Early symptoms of GPA include the following:

• A runny, stuffed-up nose
• Frequent sinusitis
• A lingering fever
• Night sweats
• Fatigue
• Nose bleeds
• Chronic ear infections
• Joint pain
• Loss of appetite, weight loss
• Blood in the urine
• Eye redness, pain or vision problems
• Chest pain
• Coughing and bloody phlegm
• Shortness of breath and wheezing
• Weakness
• Skin sores and rashes

Call your primary care doctor if any of these symptoms don't go away or get better with over-the-counter medicines. A blood test can be performed to see if you have anti-neutrophil cytoplasmic antibodies, which are present in the blood of most people with GPA.

If you are diagnosed with GPA, or your doctor suspects you have it, you will benefit from the expertise of a rheumatologist who specializes in autoimmune and musculoskeletal conditions.

GPA Treatment at Rush

Rush rheumatologists care for hundreds of patients each year with GPA and other types of vasculitis. As a result, we have a deep understanding of how this disease can affect you.

Depending on your symptoms, we may order various tests to determine the severity of your GPA and which of your blood vessels and organs are affected:

• Blood tests to look for markers of inflammation (for example, a high amount of C-reactive protein) and to see if you have anemia, which is common in GPA
• Urine tests to look for signs of kidney disease
• Imaging, such as a CT scan or MRI to look for blood vessel and organ involvement
• Chest X-rays to examine your lungs
• A biopsy to get a sample of your lungs, skin, kidneys or nerves for analysis

Your treatment will involve taking immunosuppressant medications to stop your immune system from attacking your blood vessels. For the first three to six months of your treatment (the induction phase), you will take one or more medications to suppress your immune system and control inflammation:

• Corticosteroids, such as prednisone
• Cytoxan
• Rituxan, which is given by infusion by our nurses

The maintenance phase of your treatment lasts two years or more. You will taper off of the above medications and possibly add other drugs to help to control the disease, such as the following:

• Methotrexate
• Azathioprine
• Mycophenolate

Your care team may also give you other medicines:

• Bisphosphonate to prevent bone loss caused by some steroids
• Trimethoprim or sulfamethoxazole to prevent lung infection

With treatment, most patients recover; however, GPA returns in about half of all patients. In these cases, the disease usually comes back within two years of stopping treatment.

Rush Excellence in Granulomatosis With Polyangiitis

• Leading-edge approaches: Rheumatologists at Rush conduct clinical trials on new treatments for all types of vasculitis, including GPA. This means you will have access to promising treatments as soon as they become available.
• Collaboration with other specialties: If your lungs or kidneys have been affected by GPA, you may require care from a kidney or lung doctor. Rush's kidney and pulmonary programs are recognized among the best in the nation by U.S. News & World Report. Your rheumatologist will work closely with these and other specialists at Rush to ensure your care is comprehensive and coordinated.
• Monitoring for medication side effects: The immunosuppressive medications you take to treat GPA can cause side effects, such as a higher risk of infection and bone loss. You may also experience weight gain, mood changes or other unpleasant changes. We will work with you to prevent or reduce these side effects. For instance, we may try alternative medications or lower the dose you are taking. We also prescribe preventative treatments, such as bisphosphonate to prevent bone loss.
• Compassionate, empathetic care: Treatment for GPA lasts two or more years. Our rheumatologists will be here for you the entire time, listening to your symptoms and responding to your concerns.