医学
肉芽肿伴多发性血管炎
嗜酸性
内科学
显微镜下多血管炎
抗中性粒细胞胞浆抗体
病理
血管炎
胃肠病学
皮肤病科
疾病
作者
Emma Rubenstein,Carla Maldini,Augusto Vaglio,Federica Bello,Jan Phillip Bremer,Frank Moosig,Paolo Bottero,Alberto Pesci,Renato Alberto Sinico,Julian Großkreutz,Claudia Feder,David Saadoun,Giorgio Trivioli,Federica Maritati,Barbara Rewerska,Wojciech Szczeklik,Paolo Fraticelli,Giuseppe Guida,Gina Gregorini,Gianluca Moroncini,Bernhard Hellmich,Jochen Zwerina,Matthieu Resche‐Rigon,Giacomo Emmi,Thomas Neumann,Alfred Mahr
标识
DOI:10.3899/jrheum.2023-0325
摘要
Objective Previous studies suggested that distinct phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) could be determined by the presence or absence of antineutrophil cytoplasmic antibodies (ANCA), reflecting predominant vasculitic or eosinophilic processes, respectively. This study explored whether ANCA-based clusters or other clusters can be identified in EGPA. Methods This study used standardized data of 15 European centers for patients with EGPA fulfilling widely accepted classification criteria. We used multiple correspondence analysis, hierarchical cluster analysis, and a decision tree model. The main model included 10 clinical variables (musculoskeletal [MSK], mucocutaneous, ophthalmological, ENT, cardiovascular, pulmonary, gastrointestinal, renal, central, or peripheral neurological involvement); a second model also included ANCA results. Results The analyses included 489 patients diagnosed between 1984 and 2015. ANCA were detected in 37.2% of patients, mostly perinuclear ANCA (85.4%) and/or antimyeloperoxidase (87%). Compared with ANCA-negative patients, those with ANCA had more renal ( P < 0.001) and peripheral neurological involvement ( P = 0.04), fewer cardiovascular signs ( P < 0.001), and fewer biopsies with eosinophilic tissue infiltrates ( P = 0.001). The cluster analyses generated 4 (model without ANCA) and 5 clusters (model with ANCA). Both models identified 3 identical clusters of 34, 39, and 40 patients according to the presence or absence of ENT, central nervous system, and ophthalmological involvement. Peripheral neurological and cardiovascular involvement were not predictive characteristics. Conclusion Although reinforcing the known association of ANCA status with clinical manifestations, cluster analysis does not support a complete separation of EGPA in ANCA-positive and -negative subsets. Collectively, these data indicate that EGPA should be regarded as a phenotypic spectrum rather than a dichotomous disease.