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Wegener's Granulomatosis

Table of Contents

The history of Wegener's Granulomatosis

In 1931, Heinz Klinger of the University of Berlin first reported two patients who died having prolonged sepsis with inflammation of blood vessels scattered throughout the body. Five years later, Friederic Wegener in Breslau described a distinct syndrome in three patients. These patients were found to have necrotizing granulomas involving the upper and lower respiratory tract. In 1954, seven more patients were described. This resulted in the establishment of the definite criteria for the diagnosis of the disease described by Wegener. Dr. Friederic Wegener died in July of 1990 at the age of 83.

What causes Wegener's Granulomatosis ?

The cause of Wegener's Granulomatosis remains unknown. Though the disease resembles an infectious process, no causative agent has been isolated. Anti-Neutrophilic Cytoplasmic Antibody (ANCA) is found in the majority of patients, and its level appears to correlate with the disease activity.

The Incidence of Wegener's Granulomatosis

Wegener's Granulomatosis is a quite rare disease, especially in Europe and in dark people (africans, south-americans, asian people). The exact number of patients is not known, but a very rough estimate is two new cases per million Americans per year, or about 500 new cases diagnosed every year in America.

What are the symptoms of Wegener's Granulomatosis ?

The symptoms of Wegener's Granulomatosis, and the severity of those symptoms, vary from one patient to another, although most patients first notice symptoms in the upper respiratory tract. A common manifestation of the disease is a persistent rhinorrhea ("runny nose") or other cold-like symptoms that do not respond to standard treatment, and that become progressively worse. Rhinorrhea can result from sinus drainage and can cause upper respiratory obstruction and pain. Complaints include discharge from the nose, sinusitis, nasal membrane ulcerations and crusting, inflammation of the ear with hearing problems, cough, coughing of blood and pleuritis (inflammation of the lining of the lung).

Other initial symptoms include fever, fatigue, malaise (feeling ill), loss of appetite, weight loss, joint pain, night sweats, changes in the color of urine, weakness. Be aware that not all Wegener's patients experience all of the above symptoms, and that the severity of the disease is different with each patient. Fever is often present, sometimes resulting from bacterial infection in the sinuses.

One third of patients may be without symptoms at the onset of the disease.

How do you diagnose Wegener's Granulomatosis ?

Laboratory tests are not specific for Wegener's Granulomatosis and only suggest that that the patients has an inflammatory disease. Blood tests often show anemia (low red blood cell count) and other changes in the blood. Chest X-rays and kidney biopsy are important tools used in diagnosing Wegener's Granulomatosis. For the most effective treatment, early diagnose is critical.

Asymptomatic patients can be diagnosed by ANCA blood tests and CT scans of sinuses and lungs.

It takes 5-15 months, on average, to make a diagnosis of Wegener's Granulomatosis. 40% of all diagnosis are made within less than 3 months, 10% within 5-15 years.

Other diagnositic tools are as follows:



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