Granulomatosis with polyangiitis

  • Definition
    • Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener's granulomatosis.

  • Alternative Names
    • Formerly: Wegener's granulomatosis

  • Causes
    • GPA mainly affects blood vessels in the nose, sinuses, ears, lungs, and kidneys. Other areas may also be affected in some cases. The disease can be severe and prompt treatment is important.

      The exact cause is unknown, but it is an autoimmune disorder.

      GPA is most common in middle-aged adults of northern European descent. It is rare in children, but has been seen in infants as young as 3 months old.

  • Symptoms
    • Frequent sinusitis is the most common symptom. Other early symptoms include a fever that has no clear cause, night sweats, fatigue, and a general ill feeling (malaise).

      Other common symptoms may include:

      • Chronic ear infections
      • Respiratory such as nose bleeds, pain, and sores around the opening of the nose
      • Cough with or without blood in the sputum .
      • Chest pain and shortness of breath as the disease progresses
      • Loss of appetite and weight loss
      • Skin changes such as bruises and ulcers of the skin
      • Kidney problems
      • Bloody urine
      • Eye problems ranging from mild conjunctivitis to severe swelling of the eye.

      Less common symptoms include:

      • Joint pain
      • Weakness
      • Abdominal pain
  • Exams and Tests
    • You may have a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA). These are often found in people with active GPA. However, this test is sometimes comes back negative even in people with the condition.

      A chest x-ray will be done to look for signs of lung disease.

      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 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. You may have one of the following:

      Other tests that may be done include:

  • Treatment
    • You may be treated glucocorticoids (such as prednisone). These are given along with other medicines that slow down the immune response.

      Medicines used with prednisone to treat GPA include:

      • Rituximab (Rituxan)
      • Azathioprine (Imuran)
      • Cyclophosphamide (Cytoxan)
      • Methotrexate

      These medicines are effective in severe disease but they may cause serious side effects. Talk to your health care provider about your treatment plan.

      Other medicines used for the condition include:

      • Medicines to prevent bone loss caused by prednisone
      • Folic acid or folinic acid, if you are taking methotrexate
      • Antibiotics to prevent lung infections
  • Support Groups
    • Support groups with others who suffer from similar diseases may help people with the condition and their families learn about the diseases and adjust to the changes associated with the treatment.

  • Outlook (Prognosis)
    • Without treatment, people with severe forms of this disease can die within a few months.

      With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better.

      The disease may return about half the time. It often comes back within 2 years of stopping treatment, so ongoing follow-up is needed.

  • Possible Complications
    • Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement 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 possibly death occur in most cases.

      Other complications may include:

      • Eye swelling
      • Lung failure
      • Coughing up blood
      • Nasal septum perforation (hole inside the nose)
      • Side effects from medicines used to treat the disease
  • When to Contact a Medical Professional
    • Call your health care provider if:

      • You develop chest pain.
      • You cough up blood.
      • You have blood in your urine.
      • You have other symptoms of this disorder.
  • Prevention
    • There is no known prevention.

  • References
    • Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Ann Rheum Dis. 2011;70(4):704. PMID: 21372195

      Savage COS, Harper L. Antineutrophil Cytoplasm Antibody Associated Vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 89.

      Stone JH. The systemic vasculitides. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 270.

      Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221. PMID: 20647199