Wegener's Granulomatosis

What is Wegener's Granulomatosis?

Wegener's granulomatosis is a rare disease characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body. Wegener's granulomatosis mainly affects the upper respiratory tract, lungs, and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart, and skin.

Wegener's granulomatosis can affect people of all ages from childhood to adulthood. It affects men and women equally.

Symptoms of Wegener's Granulomatosis

The symptoms of Wegener's granulomatosis and their severity vary among patients. General signs of the disease may include:

Loss of appetite
Weight loss
Fever
Fatigue

Most patients first notice symptoms in the respiratory tract. Symptoms may include:

Persistent runny nose or the formation of nasal crusts and sores
Nasal or facial pain
Nose bleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
Cough which may include bloody phlegm caused by upper airway or lower airway (lung) inflammation
Chest discomfort
Middle ear inflammation (also called otitis media), pain, or hearing loss
Voice change, wheezing, or shortness of breath caused by inflammation of the trachea

Causes of Wegener's Granulomatosis

The cause of Wegener's granulomatosis is unknown.

Diagosing Wegener's Granulomatosis

Wegener's granulomatosis has symptoms similar to a number of other disorders, which may make it difficult to diagnose. However, for the most effective and successful treatment, early diagnosis is critical.
It is the combination of symptoms, results of physical examinations, laboratory tests, x-rays and sometimes a biopsy (sample) of affected tissue (skin, nose, sinus, lung, or kidney) that together prove the diagnosis of Wegener's granulomatosis. Following treatment, these factors are also critical in judging whether the disease is active or in remission.

A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not by itself prove the diagnosis of Wegener's granulomatosis or determine disease activity.

Other tests that influence a physician's judgement of disease activity include:

Measures of anemia (red blood cell count)
Sedimentation rate (the speed in which blood cells settle in a vertical glass tube)
Urinalysis
Chest or sinus x-rays

Sometimes the lungs may become abnormal even though there are no symptoms, such as cough or shortness of breath. Therefore, it is important to periodically have lung x-rays if you are diagnosed with Wegener's granulomatosis, even if you don't have any symptoms of lung disease.

Treating Wegener's Granulomatosis

Because Wegener's granulomatosis is often a life-threatening disease, it is treated with a variety of powerful medications that have been shown to be life-saving. Treatment usually includes corticosteroid medications such as Prednisone and chemotherapy drugs such as cyclophosphamide or methotrexate.
These drugs suppress the immune system and usually induce remission (the complete absence of all signs of the disease). Improvement usually occurs within days to weeks.

When the disease is in remission, patients will reduce the dosage of these medications, but will continue treatment until the disease has been in continuous remission for one year.