http://my.clevelandclinic.org/disorders/wegener_granulomatosis/hic_wegeners_granulomatosis.aspx

What is Wegener's granulomatosis?

Wegener's granulomatosis is a rare disease of uncertain cause. It is characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body.

Wegener's granulomatosis primarily affects the upper respiratory tract [sinuses, nose, trachea (upper air tube)], lungs, and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart, and skin.

What are the symptoms?

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 (also called rhinorrhea) 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 that might 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

Other possible symptoms include:

Eye inflammation
Joint pain (arthritis) or muscle pain
Rashes or skin sores
Kidney inflammation*

*Although kidney inflammation is common, it is not usually associated with symptoms, such as pain.

How is it diagnosed?

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 doctor's judgment 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.

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