Wegener's Granulomatosis

Tuesday, January 5, 2010

Wegener's granulomatosis is a disease in which blood vessels get inflamed and blood does not flow well, as it should. This rare medical condition is a type of vasculitis that affects the kidneys and other organs. Due to its end-organ damage, Wegemer's granulomatosis can be a serious and requires long-term immunosuppression. The disease is named for Dr. Friedrich Wegener, who described the malady in 1936.

The cause of Wegener's granulomatosis is not known yet. It could be an autoimmune disorder. It is most common in middle-aged adults. Wegener's granulomatosis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-size blood vessels.

The first symptoms of Wegener's granulomatosis varies widely. So, diagnosis can sometimes be severely delayed becauses of the nonspecific nature of the symptoms. Rhinitis is generally the first sign in most patients. Upper airway, eye and ear disease. Nose: pain in the nose, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity due to a perforated septum; ear: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (unclear mechanism); strawberry gingivitis, underlying bone destruction with loosening of teeth, non-specific ulcerations throughout oral mucosa; eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis.

But the disease becomes dangerous when it affects the kidney and lungs. The renal symptom of the Wegener's granulomatosis is rapidly progressive glomerulonephritis (75%), which leads to chronic renal failure. The lungs develop pulmonary nodules, which are often referred to as "coin lesions", infiltrates, which are often interpreted as pneumonia, cavitary lesions, pulmonary hemorrhage causing hemoptysis, and rarely bronchial stenosis.

Treatment for Wegener's granulomatosis

Before steroid treatment became available, mortality within one year was over 90%, with average survival being 5 months. Steroids prolonged average survival to 8 months. The introduction of cyclophosphamide (CYC) in the 1970s was a major breakthrough. Initial treatment is generally with corticosteroids and oral CYC, 1 mg/kg/day and 2 mg/kg/day, respectively. Occasionally CYC is given in monthly intravenous (IV) doses. Monitoring of the white blood count is essential during CYC therapy. Once remission is attained (normally 3 to 6 months), treatment is frequently changed to azathioprine or methotrexate, which are less toxic drugs. Total duration of therapy should be at least one year, or longer in high risk patients. Corticosteroids are tapered to a low maintenance dose, 5–10 mg/day. Plasmapheresis may be beneficial in severe disease or pulmonary hemorrhage.