A rare disorder in which granulomas (nodular collections of abnormal cells), associated with areas of chronic tissue inflammation due to vasculitis, develop in the nasal passages, lungs, and kidneys. It is thought that the condition is an autoimmune disorder (in which the body’s natural defences attack its own tissues). Principal symptoms include a bloody nasal discharge, coughing (which sometimes produces bloodstained sputum), breathing difficulty, chest pain, and blood in the urine. There may also be loss of appetite, weight loss, weakness, fatigue, and joint pains.
Treatment is with immunosuppressant drugs, such as cyclophosphamide or azathioprine, combined with corticosteroids to alleviate symptoms and attempt to bring about a remission.
With prompt treatment, most people recover completely within about a year, although kidney failure occasionally develops.
Without treatment, complications may occur, including perforation of the nasal septum, causing deformity of the nose; inflammation of the eyes; a rash, nodules, or ulcers on the skin; and damage to the heart muscle, which may be fatal.
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