Home > Diseases and Conditions > Wegener’s granulomatosis
  • We are sorry, but NCBI web applications do not support your browser and may not function properly. More information

PubMed Health. A service of the National Library of Medicine, National Institutes of Health.

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

A.D.A.M. Medical Encyclopedia.

Wegener’s granulomatosis

Midline granulomatosis

Last reviewed: February 8, 2011.

Wegener's granulomatosis is a rare disorder in which blood vessels become inflamed, making it hard for blood to flow.

Causes, incidence, and risk factors

Wegener's granulomatosis mainly affects blood vessels in the nose, sinuses, ears, lungs, and kidneys, although other areas may be involved.

The cause is unknown. It is thought to be an autoimmune disorder.

Wegener's granulomatosis is most common in middle-aged adults. 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 continues without an obvious 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 there is no one typical lesion associated with the disease.

There may be symptoms of kidney disease. The urine may be bloody.

Eye problems develop in many people with Wegener's granulomatosis. The eye problems range from mild conjunctivitis to severe swelling of the eye.

Other symptoms include:

  • Chest pain
  • Cough, with or without blood
  • Joint pain
  • Shortness of breath
  • Weakness

Signs and tests

Your doctor may order a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA), which are often found in people with active Wegener's granulomatosis. However, it is not foolproof -- the test may be negative in some patients.

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 better 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 Wegener's granulomatosis include:

These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor.

Other medicines may be prescribed, including:

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.

Expectations (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.

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

Complications

Complications usually result from lack of treatment. Patients with Wegener's granulomatosis develop sores (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 more than 90% of patients.

Other complications may include:

  • Eye swelling
  • Lung failure
  • Nasal septum perforation (hole inside the nose)
  • Side effects from medicines used to treat the disease

Calling your health care provider

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. Channick RN, Rubin LJ. Pulmonary Vaculitis and Primary Pulmonary Hypertension. In: Mason RJ, Murray JF, Broaddus VC, Nadel JA, eds. Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: Saunders Elsevier; 2005: 1462-1463.
  2. Calabrese LH, Molloy ES, Duna G. Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. In: Firestein GS, Budd RC, Harris ED Jr., McInnes IB, Ruddy S, eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008: Chap 82.

Review Date: 2/8/2011.

Reviewed by: Andrew Schriber, MD, FCCP. Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, 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.

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 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.
See all (2) ...

Figures

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

PubMed Health Blog...

read all...

MedlinePlus.gov links to free, reliable, up-to-date health information from the National Institutes of Health (NIH) and other trusted health organizations.

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...