Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) often begins with inflammation of small- and medium-sized blood vessels and tissues in the nose, sinuses, throat, lungs, or kidneys.
Granulomatosis with polyangiitis is most common among whites but can occur in all ethnic groups and at any age. Most people are affected at about age 40. Its cause is unknown. It resembles an infection, but no infecting organism has been identified. Collections of immune cells that cause inflammation (called granulomas) form nodules and ultimately destroy normal tissue. Granulomatosis with polyangiitis is often life threatening.
The disorder may begin suddenly or gradually. Usually, the first symptoms involve the upper respiratory tract—the nose, sinuses, ears, and windpipe (trachea). They may include the following:
Sometimes only the upper respiratory tract is affected for many years. People may also have a fever, feel generally unwell, and lose their appetite. Inflammation can affect the eyes, which may become swollen, red, and painful.
The disorder may progress to affect other areas of the body or may affect several organs from the beginning:
Anemia is common and can be severe.
Granulomatosis with polyangiitis must be diagnosed and treated early to prevent complications, including kidney disorders, lung disorders, and heart attacks.
Doctors usually suspect the diagnosis based on the distinctive pattern of symptoms. For example, doctors suspect the diagnosis when people have unexplained respiratory symptoms (including otitis media in adults, which is otherwise unusual) particularly if problems in other organs, especially the kidneys, exist. Doctors also suspect the diagnosis in people who have a long history of sinus problems that have not been cured by or only partially cured by antibiotics.
A chest x-ray is done because the lungs are usually affected. However, symptoms and chest x-rays can resemble those of several lung disorders, making the diagnosis difficult. For example, a chest x-ray may show cavities or dense areas in the lungs that look like cancer or infection.
Although blood test results cannot specifically identify granulomatosis with polyangiitis, they can strongly support the diagnosis. One such test can detect antineutrophil cytoplasmic antibodies in the blood. These antibodies attack certain white blood cells and occur in several types of vasculitis. Other specific antibodies are identified with further testing. People with this disorder may have blood and protein in their urine due to kidney inflammation. Because kidney inflammation can cause damage without causing symptoms, doctors always test urine.
Doctors can confirm the diagnosis by examining a small piece of tissue under a microscope (biopsy). The tissue sample may be taken from an affected area, such as the nasal passages, airways, or lungs. Skin and kidney biopsies may occasionally be helpful.
The prognosis depends on how widespread the disorder is, how much organ damage has occurred, and how quickly people receive treatment.
For people with severe disease, prognosis has been greatly improved by using drugs that suppress the immune system (immunosuppressants). With treatment, symptoms completely disappear (called remission) for about 70% of people. However, in about half of treated people, symptoms return (called a relapse). Relapses may occur when treatment is stopped or many years later. Resuming or increasing treatment can usually control the disorder. In the past, 90% of people had some permanent harmful side effects caused by the disorder or the treatments.
Corticosteroids (such as prednisone) are almost always used to suppress inflammation. Most people also need immunosuppressants, such as cyclophosphamide, methotrexate, rituximab, or azathioprine. If the disorder is not severe, doctors give a corticosteroid and methotrexate. Rituximab may be given instead of methotrexate. If the disorder is severe, doctors give high doses of a corticosteroid and cyclophosphamide or rituximab. A plasma exchange (plasmapheresis) may be done occasionally. Most people feel better within days to weeks. But for some, improvement may take months.
During remission, the dose of the drugs is gradually reduced. Treatment is usually continued for at least a year or often for many years after the symptoms disappear. The dose of a corticosteroid can usually be gradually decreased and eventually stopped. Doses may need to be adjusted throughout the course of treatment. If symptoms worsen or recur, the dose is increased, or if drugs have been stopped, they are started again.
Because immunosuppressants weaken the immune system, the risk of developing serious infections is increased. Taking prednisone for a long time can result in weight gain, cataracts, high blood pressure, decreased bone density, diabetes, changes in mood, and difficulty sleeping. Cyclophosphamide can cause bladder irritation and, if taken repeatedly, sometimes bladder cancer. When cyclophosphamide is given by vein (intravenously), mesna, which chemically counteracts some toxic effects of cyclophosphamide on the bladder, is also given. A complete blood count is done frequently, sometimes as often as once a week for patients who take potent immunosuppressants. Immunosuppressants may cause the number of blood cells to decrease.
People with granulomatosis with polyangiitis need to be closely monitored by their doctor to check whether the dose of the drugs is appropriate, whether they are having side effects from the drugs, whether they could have an infection, and, during remission, whether there is any indication of a relapse.
People should also learn as much as they can about the disorder. Thus, they can recognize signs of a relapse early. People can also learn to test their urine for blood and protein so that they can notify their doctor at the first sign of any new abnormality.
If people develop chronic kidney disease with kidney failure, they may need a kidney transplant.
Last full review/revision April 2013 by Carmen E. Gota, MD