Granulomatosis with Polyangiitis 

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare form of vasculitis. In this disorder, small blood vessels in the nose, sinuses, ears, lungs and kidneys become inflamed and narrowed. Narrowed blood vessels prevent blood and oxygen from reaching your tissues and organs. The disease can produce a type of inflammatory tissue known as a granuloma. Granulomas can destroy normal tissue.  

GPA is most common in middle-aged adults, with an average age of onset between 40 and 65 years. It is rare in children, but has been seen in infants as young as 3 months old. Whites are more likely than blacks to develop GPA. 

The exact cause of granulomatosis with polyangiitis is unknown, but it is an autoimmune disorder. An autoimmune disorder occurs when the body’s immune system attacks and destroys healthy body tissue by mistake. GPA is not contagious and it is not inherited from your parents. 

Sinus and lung symptoms are most common in GPA. Other early symptoms include fever, night sweats, fatigue and a general ill feeling (malaise). For some people, the disease is limited to the respiratory system and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms early in the course of the disease. It may be detected only by blood and urine tests. Over time, kidney failure and anemia often occur. 

Signs and symptoms may include: 

• Sinus pain and inflammation.
• Runny nose, with pus-filled discharge.
• Nosebleeds.
• Fever.
• General ill feeling (malaise).
• Unintended weight loss.
• Ear infections.
• Cough.
• Chest pain.
• Coughing up blood.
• Shortness of breath.
• Joint aches and swelling.
• Blood in urine.
• Skin sores.
• Eye redness, burning or pain.

A doctor can tentatively diagnose GPA based on a combination of symptoms, physical examination results, laboratory tests and X-rays. A biopsy will usually be used to confirm it. 

The following tests may be performed: 

• ANCA. A blood test looks for proteins called antineutrophil cytoplasmic antibodies (ANCA), which may be present in people with this condition. 
• Urinalysis. Signs of kidney disease can be detected in the urine. 
• Biopsy. A small sample of affected tissue will be taken (from the skin, nasal membranes, sinuses, lungs, kidneys or other sites) and looked at under a microscope to confirm the diagnosis. 
• Imaging. CT scans, x-rays or MRIs may be done to help determine which organs are involved. They can also help monitor you're response to treatment. 

Getting treated quickly is essential because GPA can be very dangerous. With treatment, however, the outlook for most patients is good. 

Treatment includes corticosteroids to quickly reduce inflammation and medicines to slow the immune response. Medicines to treat the autoimmune response include azathioprine, cyclophosphamide and methotrexate.  

Other medicines sometimes used for the condition include bisphosphonates to prevent bone loss caused by prednisone, folic acid to combat side-effects of methotrexate and trimethoprim/sulfamethoxazole to prevent lung infections. 

In people who have very serious granulomatosis with polyangiitis, plasma exchange (plasmapheresis) can help the kidneys. This treatment filters the portion of your blood that contains disease-producing substances. You receive fresh plasma or a protein made by the liver (albumin), which allows your body to produce new plasma.  

Following the treatment program is essential in GPA because untreated – or under-treated – disease can lead to severe complications and death. The disease may return within two years of stopping treatment in about half of all patients. 

Learn about GPA, follow your doctor’s instructions, and maintain a good support system of family and friends.