Wegener's Granulomatosis

Category: Other Autoimmune Diseases Published on Wednesday, 25 June 2008 Written by Yong Tsai, MD

Over several months, Mr. Jones gradually developed a low-grade fever, sinus symptoms and a cough with unintentional weight loss with several weeks of foot numbness.  The tests his doctor ordered revealed a cavity on chest x-ray, deteriorating kidney function, a positive ANCA (antineutrophil cytoplasmic antibodies), and granulomatous vasculitis via lung biopsy.  With his accurate diagnosis of Wegener’s vasculitis, Mr. Jones was successfully treated with Cytoxan and corticosteroids.

In 1936, at the University in Breslau, Friedrich Wegener linked symptoms of granuloma and vasculi tis of the upper and lower respiratory tract with other tissues, which in 1954, became a definite criterion the disease known as Wegener’s granulomatosis (WG).  Currently, the etiology of WG is still unclear.

When the walls of certain arteries become inflamed, vasculitis occurs due to the fact that the blood vessels become narrowed, inadequately supply oxygen rich blood to tissue, thus making the tissue vulnerable for ischemia and even failure.

Even though Wegener’s granulomatosis is as serious as vasculitis, it is much less common and occurs in approximately 1 in 20,000 to 30,000 people per year, most commonly appearing between the ages of forty to fifty, but possible at any age.

Note worthily, the onset of WG may be gradual with few symptoms or sudden and vastly severe.  However, about 90% of patients seem to have shown symptoms of recurrent severe sinusitis, that fail to respond to standard therapeutic treatment, inflammation of the ear or eyes with hearing or sight complications, cough, hemoptysis (blood stained spit), fever, weight loss, fatigue, and joint pain.  Furthermore, kidney and nerve tissue are also commonly involved.

As Mr. Jones’ physician established, an accurate diagnosis of WG can be made with clinical and laboratory findings through the ANCA, blood and urine tests, chest x-ray, and tissue biopsy when needed.

Compared to 1958, when patients with WG had only an 18% chance of survival, the treatment and outcome of WG has come a long way.  In the 1970’s the biggest milestone in the treatment of WG occurred due to the emergence of cyclophosphamide (Cytoxan), which increased survival dramatically to over 90%.

Currently, based on disease activity and organ involvement, Cytoxan is used to send the WG into remission, while Methotrexate or Imuran can be prescribed later as a maintenance treatment.  And more recently, new biologic agents, such as Enbrel or Remicade, have been successful treating patients non-respondent to Cytoxan.

Unfortunately, there is no known cure for WG, however, we do know that an early diagnosis and appropriate treatment is effective to induce remission.  And even though relapses are common, they can be caught early for effective treatment and the new era of biologic agents shows only promising signs for WG.

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