Skin disorders cover a wide range of conditions, some benign, some very serious, and some even a sign of another underlying illness. A skin disorder not only affects your physical health, but also your emotional well-being.
Wegener's granulomatosis is a rare disease of uncertain cause. It is characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body. Wegener's granulomatosis primarily affects the upper respiratory tract [sinuses, nose, trachea (upper air tube)], lungs and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart and skin.
Causes of Wegener's granulomatosis
The cause is unknown. Wegener's granulomatosis is thought to be an autoimmune disorder.
Signs and symptoms of Wegener's granulomatosis
The symptoms of WG, and the severity of the symptoms, vary from patient to patient. One of the most common features is a chronic runny nose and other cold-like symptoms that do not respond to standard treatment. The cold symptoms gradually worsen and could lead to sinusitis (inflammation of the sinuses), middle ear infection ( otitis media ), cough, coughing of blood, and inflammation of the lung (pleuritis and pneumonia ). Loss of appetite and weight loss are common. Skin lesions typically occur, but there is no one typical lesion associated with the disease.
Other symptoms include fever, fatigue , loss of appetite, weight loss, joint pain , night sweats, change in urine color, and weakness.
How is Wegener's granulomatosis diagnosed?
Early diagnosis is critical for the most effective treatment of the disease. However, there are no specific laboratory tests for WG. Blood tests are used to rule out other causes of the symptoms and to determine which organs are affected. Chest x rays are used to determine if the lungs are involved. Computed tomography scans (CT scans) of sinuses and lungs, and kidney biopsy , are also important tools used in diagnosing WG.
Treatment for Wegener's granulomatosis
The most common treatment options include corticosteroid medications such as prednisone and chemotherapy drugs such as cyclophosphamide or methotrexate. The treatments are designed to suppress the immune system and usually inducethe complete absence of all signs of the disease.
Approximately half of people with Wegener's granulomatosis may experience a return of their disease. This occurs most frequently within 2 years of stopping medicine but can occur at any point both during treatment or after stopping treatment. Thus, it is extremely important that people continue to see their doctors regularly, both while they are on these medicines, as well as after the medicines have been stopped.
Because the treatment drugs suppress the immune system, there is an increased risk of developing serious infections. Prednisone can also cause weight gain, cataracts, brittle bones, high blood pressure, diabetes, and changes in mood and personality. Because the medicines used to treat Wegener's granulomatosis can have serious side effects, patients are monitored closely by their doctor. The dosage of medicine is adjusted as needed throughout the course of treatment.
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