Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body.
Formerly: Wegener’s granulomatosis
GPA mainly affects blood vessels in the nose, sinuses, ears, lungs, and kidneys. Other areas may also be affected in some cases.
The exact cause is unknown, but it is an autoimmune disorder.
Wegener's granulomatosis 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.
Chronic ear infections are common. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose.
Loss of appetite and weight loss are common. Skin changes are also common, but can take several forms.
There may be symptoms of kidney disease. The urine may be bloody.
Eye problems develop in many people with GPA. The eye problems range from mild conjunctivitis to severe swelling of the eye.
Less common symptoms include:
- Chest pain
- Cough, with or without blood
- Joint pain
- Shortness of breath
Exams and Tests
You may have a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA). These aree often found in people with active GPA. However, this test is sometimes comes back negative even in people with the condition.
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. Your doctor may suggest one of the following:
- Bronchoscopy with biopsy
- Kidney biopsy
- Nasal mucosal biopsy
- Open lung biopsy
- Skin biopsy
- Upper airway biopsy
Other tests that may be done include:
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 GPA include:
- Azathioprine (Imuran)
- Cyclophosphamide (Cytoxan)
These medicines may cause serious side effects. Talk to your doctor about your treatment plan.
Other medicines used for the condition include:
- 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.
The disease may return in about half of all patients. In these cases, the disease often comes back within 2 years of stopping treatment.
Complications most often occur when the disease is not treated. People with GPA develop tissue damage (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 most of patients.
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, coughing up blood, blood in the urine, or other symptoms of this disorder.
There is no known prevention.
Collard HR, King TE, Schwartz MI. Diffuse Alveolar Hemorrhage and Rare Infiltrative Disorders of the Lung. In: Mason RJ, Broaddus VC, Martin TR, et al. Murray & Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 60.
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: Saunders Elsevier; 2012:chap 89.
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.
Reviewed By: Allen J. Blaivas, DO, Clinical Assistant Professor of Medicine UMDNJ-NJMS, Attending Physician in the Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veteran Affairs, VA New Jersey Health Care System, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.