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Wegener's granulomatosis - Treatment

Alternative Names

Midline granulomatosis

Treatment:

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)
  • Cyclophosphamide (Cytoxan)
  • Methotrexate

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:

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.

Expectations (prognosis):

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:

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:

  • 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.

  • Reviewed last on: 2/8/2011
  • 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.

References

Channick RN, Rubin LJ. Pulmonary Vaculitis and Primary Pulmonary Hypertension. In: Mason RJ, Murray JF, Broaddus VC, Nadel JA, eds. Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: Saunders Elsevier; 2005: 1462-1463.

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.

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