Treatment

With early diagnosis and appropriate treatment, you may recover from granulomatosis with polyangiitis within a few months. You may need to keep taking prescription drugs long term to prevent relapse. Even if you're able to stop treatment, you'll need to see your doctor regularly so that he or she can continue to monitor your condition closely.

Medications

Talk with your doctor about the drug or drug combinations that are options for you and what the possible side effects are. Your treatment depends on which organs are involved and how severe your condition is. Your doctor may prescribe:

  • Corticosteroids to control inflammation. Medications such as prednisone help suppress the immune system and reduce inflammation of the blood vessels.
  • Other drugs that suppress your immune system. Several other drugs may help decrease the function of the immune system cells causing inflammation. They include cyclophosphamide, azathioprine (Azasan, Imuran) and methotrexate (Rheumatrex, Trexall).

    Rituximab (Rituxan) is another option for treating granulomatosis with polyangiitis. It may be as effective as cyclophosphamide in treating severe cases, with fewer side effects.

    Once the condition is under control, some drugs may be continued long term to prevent relapse. These include rituximab, methotrexate, azathioprine and mycophenolate (CellCept). A study including about 150 people who have granulomatosis with polyangiitis showed that those who discontinued their drugs or used lower doses had a higher relapse rate.

Side effect treatments

The drugs used to treat granulomatosis with polyangiitis have the potential to cause serious side effects, such as lowering your body's ability to fight infection. Your doctor will monitor your condition while you're taking these drugs and possibly prescribe drugs such as the following to help prevent side effects:

  • Sulfamethoxazole-trimethoprim (Bactrim, Septra) to prevent some lung infections
  • Bisphosphonates (Fosamax) to prevent bone loss (osteoporosis) associated with prednisone use
  • Folic acid, a synthetic form of the B vitamin folate, to prevent sores and other signs and symptoms associated with the depletion of folate in your body from methotrexate use

Other treatments

  • Plasma exchange. Also known as plasmapheresis, this treatment removes the liquid portion of your blood (plasma) that contains disease-producing substances. Then you receive an infusion of fresh plasma or a protein made by the liver (albumin), which allows your body to produce new plasma. In people who have very serious granulomatosis with polyangiitis, plasmapheresis can help the kidneys recover.
  • Kidney transplant. With advanced disease, you may need a kidney transplant to restore normal kidney function. Talk with your doctor to determine whether a kidney transplant might be an option for you. Your eligibility to receive a donated kidney will depend, in part, on how your other organs have been affected by the disease.