Gary S. Hoffman, MD, MS; Carol A. Langford, MD, MHS
Potential Financial Conflicts of Interest: None disclosed.
Hoffman G., Langford C.; Various Forms of Life in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Ann Intern Med. 2006;144:378-379. doi: 10.7326/0003-4819-144-5-200603070-00020
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Published: Ann Intern Med. 2006;144(5):378-379.
Hogan and colleagues have restated their belief that the 4 diseases included in their study under the rubric of “ANCA vasculitis” are “phenotypic variants of a single entity, small-vessel vasculitis.” This assertion ignores abundant evidence of important differences among these diseases that influence presentation, complications, selection of treatment strategies, and outcomes.
These disease entities have unique clinical differences. Consider, for example, the patient with Wegener granulomatosis who presents with chronic ear, nose, throat, or tracheal damage, none of which are complications of microscopic polyangiitis or renal-limited pauci-immune glomerulonephritis and rarely emerge in the context of the Churg–Strauss syndrome. The strategies to treat such complications extend beyond deciding which cytotoxic agent to add to corticosteroid therapy. In Wegener granulomatosis, one must consider whether chronic otitis media and conductive hearing loss may require placement of tympanotomy tubes, whether nasal complications require debridement of crusts or measures to improve moisturization, and whether subglottic or large bronchus stenosis requires surgical interventions. Unlike microscopic polyangiitis or renal-limited disease, active Wegener granulomatosis can also present with inflammatory mass lesions that may appear in any organ and must be differentiated from a comorbid malignancy or abscess. Although asthma and peripheral eosinophilia are infrequently found in Wegener granulomatosis, microscopic polyangiitis, and renal-limited disease, they are characteristic manifestations of the Churg–Strauss syndrome that can influence clinical decision making.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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