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Wegener’s Granulomatosis Overview

Wegener’s granulomatosis (WG) is an inflammation of the blood vessels of lungs, kidneys and other organs in the body. The disease is serious (and in some cases even systemic) and there are no recognized causes of the disease itself. The condition is life threatening because it damages all affected organs in the body.

Estimated survival of WG patients is about 87%, five years after the established diagnosis. Mortality may be caused by the disease or by the medications used for the treatment of the disease.

Patients usually consult the ophthalmologist because of the eye problems they keep experiencing and this doctor is the first one that should suspect the WG. Ophthalmologist can establish the diagnosis after he/she has seen the necrotizing scleritis and done some lab testing to confirm it.

Laboratory testing might include: blood tests, urine analysis, chest X ray, and some special testing, such as: cANCA, computerised tomography, renal function tests, nasal and orbital biopsy and c-reactive protein tests. Sometimes, necrotizing scleritis might be caused by some infections and the conjuctival scraping may be done to rule out this possibility.

In some cases, doctors might suspect that the patient is suffering from tuberculosis or rheumatoid arthritis. In those cases, the presence of progressing scleritis, orbital disease and/or corneal problems, accompanied with some multisystem health problems will indicate the Wegener’s disease.

The therapy requires the multisystem approach to every patient suffering from this disease. Corticosteroids are the first choice to treat the WG but they don’t work alone and they are not sufficient to stop the disease. Usually, doctors recommend combination of corticosteroids and immunosuppresives to control the progression of Wegener’s granulomatosis. The most commonly used medications are intravenous methyl-prednisolone and cyclophosphamide, and after that oral use of prednisolone and cyclophosphamide.

Potential Health Problems

Wegener’s granulomatosis may cause problems with many organs in the human organism. The eyes, complete respiratory tract and the kidneys are most commonly affected organs, but there are also case of isolated lung or airways complications (in limited form of the WG). If the patient is suffering from the complete form of the WG, there is usually necrotising granuloma of the respiratory tract, focal necrotising glomerulonephritis and systemic vasculitis.

About 29 to 79% of WG patients might experience ocular problems. They might be caused by the progression from the paranasal sinuses or be the result of focal vasculitis. Eye problems related to this disease include: necrotizing scleritis, peripheral keratopathy and orbital problems as the most common symptoms in WG patients. If the disease is mild, patients might experience nodular or diffuse scleritis. More serious complications are: fistulas, kerato-conjunctivitis sicca, retinitis, retinal vascular occlusions, nasolacrimal obstructions, uveitis, exudative retinal detachments optic neuritis, and orbital pseudotumour.

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