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A.D.A.M. Medical Encyclopedia [Internet]. Atlanta (GA): A.D.A.M.; 2013.

A.D.A.M. Medical Encyclopedia.

Wegener’s granulomatosis

Formerly: Wegener’s granulomatosis

Last reviewed: March 3, 2013.

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.

Causes

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.

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).

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:

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:

Other tests that may be done include:

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 GPA include:

These medicines may cause serious side effects. Talk to your doctor about your treatment plan.

Other medicines used for the condition include:

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.

Outlook (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.

The disease may return in about half of all patients. In these cases, the disease often comes back within 2 years of stopping treatment.

Possible Complications

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:

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.

Prevention

There is no known prevention.

References

  1. 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.
  2. 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.
  3. 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. [PubMed: 21372195]

Review Date: 3/3/2013.

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.

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The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

What works?

  • Intravenous immunoglobulin in addition to standard treatments for Wegener's granulomatosisIntravenous immunoglobulin in addition to standard treatments for Wegener's granulomatosis
    Wegener's granulomatosis is a rare disorder that causes inflammation of the blood vessels. This inflammation restricts blood flow to various organs which can eventually damage the organs. Organs most affected by Wegener's include the lungs, upper respiratory tract, kidneys, joints, skin and eyes. Wegener's granulomatosis also produces a granuloma (a mass or nodule of inflammatory tissue) which is found around the blood vessels and which can also damage surrounding tissue. The cause of Wegener's granulomatosis is unknown. Treatment is with corticosteroids and cytotoxic drugs which are often used for chemotherapy. Most patients get better with these drugs. However, the disorder returns in approximately half of patients. Intravenous immunoglobulin (IVIg) is an expensive and fairly rare blood product that has been used to treat Wegener's granulomatosis but its effects on the disorder are unknown. We asked if IVIg provided an advantage as an additive to standard treatments. We found one small randomized trial in which 34 participants were randomized to receive IVIg or placebo once daily in addition to azathioprine and prednisolone for remission maintenance. This trial did not provide enough evidence to determine if IVIg has an advantage over corticosteroids and immunosuppressants for the treatment of Wegener's granulomatosis.
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Figures

  • Adenocarcinoma - chest x-ray.
    Wegener's granulomatosis on the leg.
    Respiratory system.

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