WEGENER'S GRANULOMATOSIS

. Posted in SPECIAL PATIENTS

Wegener’s granulomatosis affects the respiratory tract from the nose and sinuses to the lungs. It also affects the kidneys. This disease is a systemic inflammatory disease whereby the body creates autoantibodies: antibodies that attack your own organs and body structures. In the nose, sinuses, and face this can lead to a collapse of the sinus structures and your nose. Patients with this disorder also have recurrent sinus infections. One of the primary symptoms is thick crusting with infection in the nasal passageway. Patients might also complain of a foul smell, bleeding, pus, and hard rock-like crusts. These patients can also experience swelling of their salivary glands, but this is rare.

The diagnosis is established by a tissue biopsy by an otolaryngologist. Blood testing can also reveal an elevated erythrocyte sedimentation rate and a positive anti-proteinase 3 antibody titer by enzyme-linked immunoabsorbent assay (ELISA) methodology. These are the blood tests that your physician should order if he or she believes that you suffer from Wegener’s.

The treatment of this disorder is varied and includes chemotherapy with cytotoxic medication as well as steroids in addition to various immunosuppressives and different monoclonal antibodies. It is often difficult to keep the nose clean, so local care with frequent irrigation as well as an emollient is critical. Surgical treatment is generally geared on a symptomatic level, relieving pus, pain and pressure; and sinus surgery may be necessary to open up the areas to make them more accessible to relieve the crusting and infection.

For people with nasal and facial collapse, plastic surgery with grafting has become fairly successful. When I was consulting at the National Institutes of Health, we did research on different grafting techniques because of the poor success such patients had been experiencing. Dr. Robert Lebovics and I pioneered the use of certain types of grafts that
have given patients new hope. These reconstruction efforts have met with much greater success than older procedures. Many patients experience problems with chronic eye tearing and require stents or even reconstruction of the tear ducts. Neodymiumyttrium-aluminum-garnet (YAG) laser helps burn through the bone via an intranasal approach, thus limiting the need for external surgery to repair the tear ducts. The repair of the tear ducts is known as dacryocystorhinostomy.

Churg-Strauss vasculitis is another disease that is similar to Wegener’s. This disease is usually present with asthma and, necrotizing granulomata and vasculitis. Kidney disease is not present in Churg - Strauss, but Wegener’s granulomatosis can cause renal failure. This can lead to decreased or absent urine production, and if left untreated, can cause mental status changes and even death. The signs, symptoms, and treatment of the sinonasal tract are similar with both Wegener’s and Churg-Strauss. In Churg Strauss, people will also suffer from asthma, allergies, and have high eosinophilic counts on blood testing. In Wegener’s, patients complain of shortness of breath, spitting up blood, pneumonia, and fever. A proper medical history is critical to differentiate between the two conditions.

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