Wegener’s granulomatosis is a rare disorder in which blood vessels become inflamed, making it hard for blood to flow.
Causes, incidence, and risk factors
Wegener’s granulomatosis mainly affects blood vessels in the nose, sinuses, ears, lungs, and kidneys, although other areas may be involved.
The cause is unknown. It is thought to be an
Wegener’s granulomatosis is most common in middle-aged adults. It is rare in children, but has been seen in infants as young as 3 months old.
Frequent sinusitis is the most common symptom. Other early symptoms include a fever that continues without an obvious cause, night sweats,
Chronic ear infections are common. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose.
Loss of appetite and weight loss are common. Skin changes are also common, but there is no one typical lesion associated with the disease.
There may be symptoms of kidney disease. The urine may be bloody.
Eye problems develop in many people with Wegener’s granulomatosis. The eye problems range from mild
Other symptoms include:
- Chest pain
- Cough, with or without blood
- Joint pain
- Shortness of breath
Signs and tests
Your doctor may order a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA), which are often found in people with active Wegener’s granulomatosis. However, it is not foolproof — the test may be negative in some patients.
- Bronchoscopy with biopsy
- Kidney biopsy
Nasal mucosal biopsy Open lung biopsy
- Skin biopsy
Upper airway biopsy
Other tests that may be done include:
Bone marrow aspiration
- Chest CT scan
Your doctor may suggest treatment with glucocorticoids (such as prednisone) which are given along with other medicines that slow down the immune response.
Medicines used to treat Wegener’s granulomatosis include:
- Azathioprine (Imuran)
These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor.
Other medicines may be prescribed, including:
Bisphosphonate (Fosamax) to prevent bone loss caused by prednisone
- Folic acid or folinic acid (leucovorin) — if you are taking methotrexate
Trimethoprim/sulfamethoxazole to prevent lung infections
Support groups with others who suffer from similar diseases may help patients and their families learn about their diseases and adjust to the changes associated with the treatment.
Without treatment, people with this disease can die within a few months.
With treatment, the outlook for most patients is good. Studies have shown that most people who receive corticosteroids and cyclophosphamide get much better.
However, the disease may return in about half of all patients. In these cases, the disease usually comes back within 2 years of stopping treatment.
Complications usually result from lack of treatment. Patients with Wegener’s granulomatosis develop sores (lesions) in the respiratory tract and the kidneys. Kidney lesions cause
If untreated, kidney failure and death occur in more than 90% of patients.
Other complications may include:
- Eye swelling
- Lung failure
- Nasal septum perforation (hole inside the nose)
- Side effects from medicines used to treat the disease
Calling your health care provider
Call your health care provider if you develop chest pain, coughing up blood, blood in the urine, or other symptoms of this disorder.
There is no known prevention.