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.
Urinalysis is done to look for signs of kidney disease such as protein and blood in the urine. Sometimes urine is collected over 24 hours to better check how the kidneys are working.
A biopsy is sometimes needed to confirm the diagnosis. The exact type of biopsy depends on which area of the body the doctor wants to look at. Your doctor may suggest one of the following:
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:
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 glomerulonephritis, which may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. Kidney function may not improve even when the condition is controlled by medicines.
If untreated, kidney failure and death occur in more than 90% of patients.
Other complications may include:
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.
Calabrese LH, Molloy ES, Duna G. Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. In: Firestein GS, Budd RC, Harris ED Jr., McInnes IB, Ruddy S, eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008: Chap 82.
Andrew Schriber, MD, FCCP. Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.