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Clinical Services at Rush Granulomatosis With Polyangiitis

Granulomatosis with polyangiitis (GPA)  
(formerly Wegener’s granulomatosis)

What causes granulomatosis with polyangiitis?
What organs are affected by granulomatosis with polyangiitis?
How is the diagnosis of granulomatosis with polyangiitis made?
What is the treatment for granulomatosis with polyangiitis?
What is the prognosis for granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (GPA) is a form of autoimmune vasculitis that affects small blood vessels. It originally was named after Friedrich Wegener, a German pathologist, who reported the first case. GPA belongs to a family of small vessel vasculitides called ANCA-associated vasculitides. These include granulomatosis with polyangiitis, microscopic angiitis and Churg-Strauss syndrome.

What causes granulomatosis with polyangiitis?

GPA is an autoimmune condition caused by the overproduction of ANCA antibodies by the immune system. What triggers the immune system to make these antibodies remains unknown. However, most researchers agree that it is probably a combination of an infection and genetic predisposition. Some infections are more common in GPA like staphyloccus infections. Also, some genetic variations like alpha-antitrypsin deficiency may predispose patients to getting GPA.

What organs are affected by granulomatosis with polyangiitis?

Granulomatosis with polyangiitis may affect multiple organs:

  • Recurrent sinusitis and/or loss of hearing
  • Inflammation and redness of the eyes
  • Rash of red spots, skin bumps or nodules (leucocytoclastic vasculitis)
  • Shortness of breath
  • Cough with occasional bloody sputum
  • Kidney failure
  • Joint pain and inflammation
  • Weakness or numbness of the extremities if nerves are involved
  • Headaches or seizures (rarely)
  • Abnormalities and loss of function of other organs

How is the diagnosis of granulomatosis with polyangiitis made?

Patient history, physical exam, and laboratory testing usually make the diagnosis of GPA. Your doctor will check the level and type of ANCA antibodies and will test your blood, urine and obtain chest X-rays and CAT scans. Often a biopsy (a piece of tissue) is needed to establish the diagnosis prior to using strong immunosuppressive medications. The organs that are often biopsied are the lungs, skin, kidneys and nerves. Even though a biopsy is an invasive procedure, with recent technologies it is relatively safe and carries minimal risks to patients.

What is the treatment for granulomatosis with polyangiitis?

GPA is treated by a class of drugs called immunosuppressives. These medications suppress the immune system and prevent it from further attacking the body and causing damage. The drugs that are often used for the treatment of GPA are: corticosteroids (prednisone or methylprednisolone), cyclophosphamide (Cytoxan), azathioprine (Imuran, Anuran), methotrexate (Methotrex), mycophenolate mofetil (Cellcept), leflunomide (Arava) and, more recently, rituximab (Rituxan). These medications require constant monitoring of the blood and urine to prevent toxicity.

The treatment of GPA consists of two phases: the “induction phase” and the “maintenance phase.” During the induction phase, strong medicines like corticosteroids and Cytoxan (pills or intravenous) or Rituxan (intravenous) are administered to completely control the disease. The induction phase may last three to six months. Later, during the maintenance phase, less-toxic drugs like methotrexate or Imuran are used to maintain disease control. This phase may last up to two years or longer.

What is the prognosis for granulomatosis with polyangiitis?

With recent advances in treatment, the current prognosis of GPA is considered to be good. Remission of the disease is usually attained in 90 percent of the cases. The sooner the diagnosis is made and the less vital organs involved, the better the outcome is.

Medication toxicity remains a major concern in granulomatosis with polyangiitis. Therefore, it is very important that patients get regular check-ups, be vaccinated and be constantly monitored for any infection or side effects of therapy. All patients affected by GPA should be under the supervision of a skilled health team that is familiar with these conditions, their treatment and the side effects of immunosuppressive medications. 

Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail

Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612

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