Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a disorder characterized by inflammation of small and middle-sized blood vessels.
The inflammation from granulomatosis with polyangiitis is so destructive that it causes lumps (called granulomas) to form in the sinuses, nose, ears, and lungs. Granulomatosis with polyangiitis can also affect small vessels in the kidneys, the skin, eyes, heart, and nervous system.
Granulomatosis with polyangiitis is a rare disorder that most commonly occurs in the 30s and 40s. It affects men and women with equal frequency.
The disorder usually develops over 4 to 12 months, with 90 percent of patients presenting with upper or lower respiratory tract symptoms, or both.
Common symptoms and signs are pain in the sinuses and ears; nasal congestion and discharge and nosebleeds. Other early symptoms include fever, fatigue, loss of appetite, weight loss, pain in the joints and muscles, skin rash, and redness and swelling in the eyes.
Other problems that may occur include a chronic cough (sometimes coughing up blood), breathing difficulties, skin ulcers, and weakness and numbness in the limbs.
A doctor will ask about symptoms and perform a physical examination. Blood tests will be done. A blood test called ANCA helps diagnosis Wegener's granulomatosis. ANCA stands for Anti-Neutrophil Cytoplasmic Antibody, which is manufactured by the body's immune system. Since most people with granulomatosis with polyangiitis produce ANCA, they usually have a positive ANCA test, especially when the symptoms worsen.
A chest X-ray or chest CT (computed tomography) scan may be done to look for masses in the lungs. Urine studies may be performed to assess kidney function.
The diagnosis often is confirmed by a biopsy (a small sample) of inflamed tissue and examination under a microscope. Usually, the biopsy is easy to get from inflamed areas in the nose or the skin, but sometimes a sample is needed from a lung or kidney.
Granulomatosis with polyangiitis is often treated with a combination of corticosteroids and immunosuppressive drugs. The corticosteroids control the inflammation, and the immunosuppressives appear to stop the growth of granulomas. The immunosuppressive drugs most commonly used to treat granulomatosis with polyangiitis are cyclophosphamide and methotrexate.
When therapy is started, corticosteroids are given in high doses. Usually, the dose can be lowered after about two to four months and gradually tapered until only the immunosuppressive drug is needed. The combination antibiotic trimethoprim-sulfamethoxazole is ineffective for life-threatening disease but has been beneficial in helping to keep patients in remission.
Both corticosteroids and immunosuppressive drugs can produce serious side effects. When a person takes high doses of corticosteroids for several months, easy bruising, osteoporosis (thinning of the bones which can lead to fractures), cataracts, weight gain, and susceptibility to infections can result.
Immunosuppressive drugs can cause anemia and other blood abnormalities, susceptibility to infections, nausea and vomiting, skin rash, sterility, and kidney and bladder problems. If a patient is taking cyclophosphamide, it is important to drink a lot of fluids (at least 3 liters or quarts of water a day) to avoid bladder infection.
Questions to ask your doctor
Are further tests such as ANCA required?
Where are the granulomas located?
What are treatment options?
Will you prescribe corticosteroids or immunosuppressive drugs?
What are side effects of these drugs?
What is the prognosis?