Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener's granulomatosis.
Formerly: Wegener's granulomatosis
GPA mainly causes inflammation of blood vessels in the lungs, kidneys, nose, sinuses, and ears. This is called vasculitis or angiitis. Other areas may also be affected in some cases. The disease can be fatal and prompt treatment is important.
In most cases, the exact cause is not known, but it is an autoimmune disorder. Rarely, vasculitis with positive antineutrophil cytoplasmic antibodies (ANCA) has been caused by several drugs including cocaine cut with levamisole, hydralazine, propylthiouracil, and minocycline.
GPA is most common in middle-aged adults of northern European descent. It is rare in children.
Frequent sinusitis and bloody noses are the most common symptoms. Other early symptoms include a fever that has no clear cause, night sweats, fatigue, and a general ill feeling (malaise).
Other common symptoms may include:
Less common symptoms include:
Because of the potentially serious nature of GPA, you may be hospitalized. Once the diagnosis is made, you will probably be treated with high doses of glucocorticoids (such as prednisone). These are given through the vein for 3 to 5 days at the beginning of treatment. Prednisone is given along with other medicines that slow down the immune response.
For milder disease other medicines that slow down the immune response such as methotrexate or azathioprine may be used.
Common medicines for vasculitis include:
These medicines are effective in severe disease, but they may cause serious side effects. Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months. Talk to your health care provider about your treatment plan.
Other medicines used for GPA include:
Peter Merkel is a Rheumatologist in Philadelphia, Pennsylvania. Merkel has been practicing medicine for over 34 years and is rated as an Elite expert by MediFind in the treatment of Granulomatosis with Polyangiitis. He is also highly rated in 21 other conditions, according to our data. His top areas of expertise are Granulomatosis with Polyangiitis, Vasculitis, Microscopic Polyangiitis, and Takayasu Arteritis. He is licensed to treat patients in Pennsylvania and Massachusetts. Merkel is currently accepting new patients.
Christian Pagnoux practices in Canada. Pagnoux is rated as an Elite expert by MediFind in the treatment of Granulomatosis with Polyangiitis. He is also highly rated in 24 other conditions, according to our data. His top areas of expertise are Granulomatosis with Polyangiitis, Vasculitis, Polyarteritis Nodosa, and Microscopic Polyangiitis.
Carol Langford is a Rheumatologist in Cleveland, Ohio. Langford has been practicing medicine for over 35 years and is rated as an Elite expert by MediFind in the treatment of Granulomatosis with Polyangiitis. She is also highly rated in 16 other conditions, according to our data. Her top areas of expertise are Granulomatosis with Polyangiitis, Vasculitis, Microscopic Polyangiitis, and Temporal Arteritis. She is licensed to treat patients in Ohio. Langford is currently accepting new patients.
Support groups with others who suffer from similar diseases may help people with the condition and their families learn about the diseases and adjust to the changes associated with the treatment.
Without treatment, people with severe forms of this disease can die within a few months.
With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better. Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months.
Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. Kidney function may not improve even when the condition is controlled by medicines.
If untreated, kidney failure and possibly death occur in most cases.
Other complications may include:
Call your provider if:
There is no known prevention.
Published Date: October 25, 2021
Published By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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