Classification of antineutrophil cytoplasmic autoantibody vasculitides: The role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis

Autor: J. Charles Jennette, Susan L. Hogan, Sophia Lionaki, Patrick H. Nachman, Ronald J. Falk, Yichun Hu, J. Brent A. Senior, Elizabeth R. Blyth, Caroline E. Jennette
Rok vydání: 2012
Předmět:
Zdroj: Arthritis & Rheumatism. 64:3452-3462
ISSN: 0004-3591
DOI: 10.1002/art.34562
Popis: The name of a disease should be informative about clinical and pathologic phenotypes, etiology and pathogenesis (when known), natural history and response to therapy. It should permit the differentiation of similar diseases that have different outcomes. Optimally, the name of a disease should reflect its underlying etiology. In 1994, the Chapel Hill Consensus Conference (CHCC) aimed to standardize nomenclature and definitions for vasculitis, including microscopic polyangiitis, Wegener’s granulomatosis, Churg Strauss syndrome, and polyarteritis nodosa.1 In 2007, the European Medicines Agency (EMA) classification system2 proposed the same disease names but different definitions that refined and expanded the 1990 American College of Rheumatology classification system.3 Since that time, granulomatosis with polyangiitis (GPA) has been proposed as an alternative term for Wegener’s granulomatosis, and will be used in place of Wegener’s granulomatosis for the remainder of this article.4 The Chapel Hill nomenclature was meant to provide disease definitions. Neither the CHCC nor EMA classification system provides diagnostic criteria for practicing physicians to discriminate microscopic polyangiitis (MPA) from GPA. The advent of widespread anti-neutrophil cytoplasmic antibody (ANCA) testing and accumulating evidence that ANCA may participate in the cause of small vessel vasculitis5 have spawned the terms ANCA associated vasculitis, ANCA vasculitis or ANCA disease as overarching terms for MPA, GPA and Kidney Limited Disease (KLD) that aid patients and clinicians in therapeutic decision-making. This approach has substantial value, yet may mask real differences in disease phenotype and prognosis unless the ANCA specificity is included in the diagnosis. We sought to evaluate the utility of three classification systems in predicting the outcomes of treatment resistance, disease relapse, end stage kidney disease (ESKD) and death in a cohort of ANCA vasculitis patients. The classification systems compared for this project were a system based on the Chapel Hill Consensus Conference (CHCC) definitions,1 the European Medicines Agency (EMA) classification system,2 and classification based on ANCA serologic specificity. We hypothesized that ANCA specificity that is anti-proteinase 3 (PR3) antibodies (PR3-ANCA) versus anti-myeloperoxidase (MPO) antibodies (MPO-ANCA), would provide a more useful classification system in distinguishing both clinical phenotype and prognosis in ANCA vasculitis than the CHCC or EMA systems alone. We also studied the added value of appending the ANCA specificity to the CHCC categories.
Databáze: OpenAIRE
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