Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's) Distinct Patient Subsets

被引:79
作者
Miloslavsky, Eli M. [1 ]
Lu, Na [1 ]
Unizony, Sebastian [1 ]
Choi, Hyon K. [1 ]
Merkel, Peter A. [2 ]
Seo, Philip [3 ]
Spiera, Robert [4 ]
Langford, Carol A. [5 ]
Hoffman, Gary S. [5 ]
Kallenberg, Cees G. M. [6 ]
St Clair, E. William [7 ]
Tchao, Nadia K. [8 ]
Fervenza, Fernando [9 ]
Monach, Paul A. [10 ]
Specks, Ulrich [9 ]
Stone, John H. [1 ]
机构
[1] Massachusetts Gen Hosp, Boston, MA 02114 USA
[2] Univ Penn, Philadelphia, PA 19104 USA
[3] Johns Hopkins Univ, Baltimore, MD USA
[4] Hosp Special Surg, 535 E 70th St, New York, NY 10021 USA
[5] Cleveland Clin Fdn, 9500 Euclid Ave, Cleveland, OH 44195 USA
[6] Univ Groningen, Univ Med Ctr Groningen, Groningen, Netherlands
[7] Duke Univ, Sch Med, Durham, NC USA
[8] Immune Tolerance Network, San Francisco, CA USA
[9] Mayo Clin, Rochester, MN USA
[10] Boston Univ, Boston, MA 02215 USA
关键词
CHURG-STRAUSS-SYNDROME; MICROSCOPIC POLYANGIITIS; POLYARTERITIS-NODOSA; VASCULITIS; CLASSIFICATION; NOMENCLATURE; POPULATION; PREVALENCE; INDEX;
D O I
10.1002/art.39812
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective. To examine the relationship of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis with demographic features, disease manifestations, and clinical outcomes. We focused on patients who account for the differences between ANCA type and disease type classifications: antimyeloperoxidase (MPO)-ANCA-positive and ANCA-negative patients with granulomatosis with polyangiitis (Wegener's) (GPA). Methods. We performed a pooled analysis of the Wegener's Granulomatosis Etanercept Trial and the Rituximab in ANCA-Associated Vasculitis trial comparing patients with MPO-ANCA-positive GPA and patients with ANCA-negative GPA to patients with proteinase 3 (PR3)-ANCA-positive GPA and patients with MPO-ANCA-positive microscopic polyangiitis (MPA). Results. Of the 365 patients analyzed, 273 (75%) had PR3-ANCA-positive GPA, 33 (9%) had MPO-ANCA-positive GPA, 15 (4%) had ANCA-negative GPA, and 44 (12%) had MPO-ANCA-positive MPA. MPO-ANCA-positive GPA patients were younger at diagnosis compared to MPO-ANCA-positive MPA patients (53 versus 61 years; P=0.02). Their disease manifestations and rates of relapse were similar to those of PR3-ANCA-positive GPA patients. Relapse was more frequent in MPO-ANCA-positive GPA patients than in patients with MPO-ANCA-positive MPA at trial entry as well as at 12 and 18 months. ANCA-negative patients with GPA had lower Birmingham Vasculitis Activity Score for Wegener's Granulomatosis scores at trial entry than PR3-ANCA-positive patients with GPA (4.5 versus 7.7; P < 0.01), primarily because of a lower prevalence of renal involvement. Conclusion. We were unable to demonstrate important clinical differences between MPO-ANCA-positive and PR3-ANCA-positive patients with GPA.
引用
收藏
页码:2945 / 2952
页数:8
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