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Various Forms of Life in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis
- Susan L. Hogan, PhD, MPH;
- Ronald J. Falk, MD;
- Patrick H. Nachman, MD; and
- J. Charles Jennette, MD
- From the University of North Carolina at Chapel Hill, Chapel Hill, NC 27599.
TO THE EDITOR:
We must disagree with some of the concepts described by Hoffman and Langford (1) in their editorial written in response to our evaluation of relapse and treatment resistance in antineutrophil cytoplasmic antibody (ANCA)–associated small-vessel vasculitis (2). The editorial primarily addressed 1) the clinical value of our results given that our cohort was not homogeneous and included patients with small-vessel vasculitis manifesting as Wegener granulomatosis, microscopic polyangiitis, and renal-limited pauci-immune necrotizing glomerulonephritis; 2) the usefulness of our requirement for ANCA seropositivity as the primary classification criterion; and 3) the risks and benefits of prolonged therapy. The authors disagreed with our suggestion that physicians should carefully weigh the risk for therapeutic complications associated with prolonged prophylactic therapy against the risk for relapse with or without therapy. This final point is one that we anticipated might generate discussion as our study challenges the current rote therapy used in this disease.
With respect to inclusion of “4 diseases as a single disease,” we contend that these are phenotypic variants of a single entity, small-vessel vasculitis. There are discrepancies in categorization of these diseases between the most commonly used definitions from the American …
