Wegener's granulomatosis: managing more than inflammation

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Abstract
Although studies of Wegener's granulomatosis frequently focus on controlling disease activity, the prevention of the chronic morbidity – known as damage – may be equally important. The first part of this review explores the concept of damage and its relevance to the diagnosis of Wegener's granulomatosis. The second part focuses on specific forms of damage associated with Wegener's granulomatosis. Some forms of damage (such as subglottic stenosis or renal insufficiency) occur as the direct result of the disease; other forms (such as osteoporosis or gonadal failure) are the result of the drugs used to treat it. There is an ongoing international effort to revise the way in which damage is measured; this initiative will benefit from longitudinal studies of vasculitis that are being conducted in the US and in Europe. Despite advances in therapeutics, renal damage in particular continues to be an important source of morbidity among patients with Wegener's granulomatosis. Recent years have also revealed previously unexpected associations between Wegener's granulomatosis and the incidence of malignancy, venous thromboembolic events, and cardiovascular disease. Damage remains an important concern for all patients with Wegener's granulomatosis. Future studies of this disease must focus on both the early identification and prevention of damage.