Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis 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 Overview of Anemia Anemia is a condition in which the number of red blood cells is low. Red blood cells contain hemoglobin, a protein that enables them to carry oxygen from the lungs and deliver it to all parts... read more 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 of granulomatosis with polyangiitis 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. So doctors also do a computed tomography (CT) scan of the chest to look for things the chest x-ray might miss, such as cavities or areas of inflammation in the lungs that may mimic 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 (c-ANCA) in the blood. These antibodies occur in several types of vasculitis and trigger certain white blood cells to attack normal organs. 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 do a urine test to detect this inflammation.

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 airways or lungs. Skin and kidney biopsies may occasionally be helpful. Sometimes, the tissue sample is taken from the nasal passages, but biopsy of nasal tissue only rarely provides a definite diagnosis.

The prognosis depends on how severe and widespread the granulomatosis with polyangiitis 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 Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more (immunosuppressants). With treatment, symptoms completely disappear (called remission) for about 80% 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.

Corticosteroids (such as prednisone) and immunosuppressants (such as cyclophosphamide, methotrexate, rituximab, or azathioprine) are used to suppress inflammation in people with granulomatosis with polyangiitis. If the disorder is not severe, doctors give a corticosteroid and methotrexate. Rituximab may be given instead of methotrexate. If the disorder is severe, people are hospitalized and are given high doses of a corticosteroid and cyclophosphamide or rituximab. 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 of granulomatosis with polyangiitis 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 return, 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.

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. A complete blood count Complete blood count Doctors select tests to help diagnose blood disorders based on the person's symptoms and the results of the physical examination. Sometimes a blood disorder causes no symptoms but is discovered... read more is done frequently, sometimes as often as once a week, for people who take potent immunosuppressants. Immunosuppressants may cause the number of blood cells to decrease.

People should also learn as much as they can about the disorder. Thus, they can recognize signs of a relapse early.

If people develop chronic kidney disease with kidney failure, they may need a kidney transplant Kidney Transplantation Kidney transplantation is the removal of a healthy kidney from a living or recently deceased person and then its transfer into a person with end-stage kidney failure. (See also Overview of Transplantation... read more .

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