Wegener’s granulomatosis (WG) is a complex multisystem vasculitic disease of unknown cause. Although once rapidly progressive and often fatal, WG is now a manageable condition in which remission can be achieved by conventional immunosuppressive therapy. With the introduction of effective therapy by Fauci and Wolff in the 1970s and 1980s, the dreaded nature of this disease was dramatically changed as investigators gained new insights into the pathogenesis and course of WG. Importantly, they had the opportunity to understand the relapsing nature of WG, appreciate the toxicities of therapy, and explore the basis for the granuloma formation and vasculitis. In 2008, it is not unusual to see people with WG who have had their illness for many years. Their course today reflects both the success made as well as the progress we have yet to make.
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Explore This IssueOctober 2008
A Case in Point
Consider the case of a 55-year-old female who, in 1991, was diagnosed with WG involving the sinuses and lungs. She was treated initially with prednisone and daily cyclophosphamide (CYC). As was the standard of care at that time, she received CYC for one year, which was then tapered and discontinued. She did well off medications for 36 months and then had a relapse involving her sinuses, peripheral nerves, and kidneys, with a peak creatinine of 2.0 mg/dL. She again received prednisone and CYC, and her creatinine normalized. After three months of treatment, she had gross hematuria, with cystoscopy revealing CYC-induced bladder injury. She was switched to azathioprine (AZA), on which she did well. After two years, while still on AZA, she experienced a sinus and lung relapse. She was taken off of AZA and treated with prednisone and methotrexate (MTX). Her disease improved but was complicated by diabetes mellitus. Over the next five years, she continued to experience sinonasal and joint relapses, for which she was kept on MTX with adjustments in her prednisone dose. She is currently in remission on MTX, but she remains diabetic and has residual sinus symptoms and neuropathic foot pain. Despite the ups and downs of her condition, she continues to enjoy her work, family, and community activities.
WG is most commonly associated with disease of the sinuses, lungs, and kidneys, but it is very much a multisystem disease.1 Other sites of involvement include the skin, joints, eyes, and peripheral or central nervous system. Pulmonary involvement can have many radiographic presentations, including single or bilateral nodules or infiltrates, cavities, or ground glass infiltrates suggestive of alveolar hemorrhage (see Figure 2, p. 21). Subglottic and endobronchial stenosis should be considered in any WG patient with an unexplained cough or dyspnea. Ninety percent of patients with WG will first present with upper and or lower airway symptoms. Because asymptomatic disease can occur in the lungs and kidneys, pulmonary imaging and urinalysis should be performed in all patients when WG is being considered. Patients with WG have an increased incidence of venous thrombotic events, making awareness of deep venous thrombosis or pulmonary embolism important.2