What is Wegener's granulomatosis?
Wegener's granulomatosis is a rare disease of uncertain cause. It is characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body.
Wegener's granulomatosis primarily affects the upper respiratory tract [sinuses, nose, trachea (upper air tube)], lungs, and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart, and skin.
Wegener’s granulomatosis usually affects the upper respiratory tract (sinuses, nose and trachea), lungs and kidneys. Not all patients will have all sites affected.
Who is affected by the disease?
Wegener's granulomatosis can affect people of all ages from childhood to adulthood. It affects men and women equally.
What are the symptoms?
The symptoms of Wegener's granulomatosis and their severity vary among patients. General signs of the disease may include:
- Loss of appetite
- Weight loss
- Fever
- Fatigue
Most patients first notice symptoms in the respiratory tract. Symptoms may include:
- Persistent runny nose (also called rhinorrhea) or the formation of nasal crusts and sores
- Nasal or facial pain
- Nose bleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
- Cough that might include bloody phlegm caused by upper airway or lower airway (lung) inflammation
- Chest discomfort
- Middle ear inflammation (also called otitis media), pain, or hearing loss
- Voice change, wheezing, or shortness of breath caused by inflammation of the trachea
Other possible symptoms include:
- Eye inflammation
- Joint pain (arthritis) or muscle pain
- Rashes or skin sores
- Kidney inflammation*
*Although kidney inflammation is common, it is not usually associated with symptoms, such as pain.
How is it diagnosed?
Wegener's granulomatosis has symptoms similar to a number of other disorders, which may make it difficult to diagnose. However, for the most effective and successful treatment, early diagnosis is critical.
It is the combination of symptoms, results of physical examinations, laboratory tests, X-rays, and sometimes a biopsy (sample) of affected tissue (skin, nose, sinus, lung, or kidney) that together prove the diagnosis of Wegener's granulomatosis. Following treatment, these factors are also critical in judging whether the disease is active or in remission.
A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not by itself prove the diagnosis of Wegener's granulomatosis or determine disease activity.
Other tests that influence a doctor's judgment of disease activity include:
- Measures of anemia (red blood cell count)
- Sedimentation rate (the speed in which blood cells settle in a vertical glass tube) Urinalysis
- Chest or sinus X-rays
Sometimes the lungs may become abnormal even though there are no symptoms such as cough or shortness of breath. Therefore, it is important to periodically have lung X-rays if you are diagnosed with Wegener's granulomatosis - even if you don't have any symptoms of lung disease.
What are the treatments?
Because Wegener's granulomatosis is often a life-threatening disease, it is treated with a variety of powerful drugs that have been shown to be life-saving.
Treatment usually includes corticosteroid medicines, such as prednisone, and chemotherapy drugs, such as cyclophosphamide or methotrexate. These drugs suppress the immune system and usually induce remission (the complete absence of all signs of the disease). Improvement usually occurs within days to weeks. When the disease is in remission, patients will reduce the dosage of these medicines, but will continue treatment until the disease has been in continuous remission for one year.
The treatment used for Wegener's granulomatosis has also been successfully applied to other vasculitic diseases.
What are the side effects of treatment?
Because the treatment drugs suppress the immune system, there is an increased risk of developing serious infections. Prednisone can also cause weight gain, cataracts, brittle bones, high blood pressure, diabetes, and changes in mood and personality. Cyclophosphamide can cause sterility, bladder irritation, and bleeding, and even cancer of the bladder. Methotrexate can cause liver irritation. Cyclophosphamide and methotrexate can each cause changes in blood counts and sometimes lung inflammation.
Because the medicines used to treat Wegener's granulomatosis can have serious side effects, patients are monitored closely by their doctor. The dosage of medicine is adjusted as needed throughout the course of treatment.
What is the outlook for people with Wegener's granulomatosis?
After treatment and improvement occurs, relapses of the disease might occur in half of all patients. Flare-ups might follow reductions of the doses of prednisone, methotrexate, or cyclophosphamide. Flare-ups can usually be controlled by increasing these medicines.
Wegener's granulomatosis is a very serious disease, and its treatment carries significant risks. However, treatment is life-saving for almost everyone when the diagnosis is timely and proper medicines have been started.
Prior to recognizing effective therapy in the 1970s, half of all patients with this illness died within five months of diagnosis. Today, more than 75 percent of treated patients are alive at least eight years later. Many of these patients have not experienced relapses and have been able to lead relatively normal lives.
