COUGH WITH WEGENER’S GRANULOMATOSIS

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What is the best treatment for persistent cough in a patient with Wegener’s granulomatosis (WG) who has a normal chest x-ray?
—James A. Skrabak, DO, Greenwood, Ind.

A patient known to have active WG should be referred to a subspecialist for appropriate treatment, most commonly with cyclophosphamide and corticosteroids. If the patient’s WG status is unknown, a clear diagnosis of the cough’s etiology should be established, given the potential toxicity of treatment and the potential progression of disease without treatment. Cough can be one of the frequent, early, lower respiratory tract manifestations (Ann Intern Med. 1992;116:488-498). However, it need not be. For example, patients with WG may also develop intercurrent or post-treatment respiratory tract infections. Patients in the midst of treatment may be at higher risk of atypical infections with such organisms as Pneumocystis due to immunosuppression. By history, you may look for other, typical upper respiratory tract involvement, such as rhinorrhea, purulent or bloody nasal discharge, or just sinus pain. Lower respiratory tract involvement may cause cough as noted, or even hemoptysis and dyspnea. WG may be “limited” to respiratory involvement in about one third of patients, or it may involve other major organ systems, such as the kidneys and skin. Lab studies might show thrombocytosis or an elevated erythrocyte sedimentation rate. The vast majority of patients with active WG will also have circulating antineutrophil cytoplasmic antibodies (Am J Med. 2004;117:39-50). A normal chest x-ray by no means excludes WG, and in some cases, chest CT may show findings absent on plain radiograph. Ultimately, tissue biopsy at a site of suspected active disease will be needed to confirm the diagnosis in the right clinical setting.
—Christopher Ruser, MD (109-6)

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