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Wegener’s granulomatosis (WG) is a necrotising granulomatous vasculitis which has a clinical predilection for the upper airways, lungs, and kidneys. The cause of WG remains unclear although recent investigations have begun to shed light on the immune mechanisms that may play a part in the pathophysiology of the disease. Therapeutic interventions in WG were largely ineffective until the introduction of combined treatment with cyclophosphamide and glucocorticoids which dramatically improved patient outcome. While disease relapse and drug toxicity remain of concern with this regimen, it has given us the opportunity for longer term follow up of patients from which we have gained a greater understanding of the disease.
Epidemiology
The prevalence of WG in the United States has been estimated to be at least three per 100 000 with a male to female ratio of about 1:1. Most patients (80–97%) are Caucasian, whereas African-Americans comprise about 2–8% of most cohorts. The mean age at diagnosis is about 40–55 years, although individuals of any age may be affected.1 Whether there is significant variation in onset of disease with different seasons or precipitation of illness by inhalation of certain particulate or gaseous materials or infection remains a matter of controversy. Recent studies have not supported the notion of seasonal variation whether the data be derived from patient perceptions of disease onset, medical records, or national hospital discharge surveys.1 ,2
A case-control study that compared patients with WG, healthy controls, individuals with idiopathic inflammatory pulmonary diseases and a variety of rheumatological diseases evaluated exposure to environmental particulate materials and gaseous substances.2 Over 75% of patients in all groups noted environmental exposure to inhaled materials during the year prior to onset of the diseases (or the interview date for healthy controls). The absence of a striking difference between patients with WG and control …