188 例日本嗜酸性肉芽肿性多血管炎患者的长期预后。
Longterm Outcomes of 188 Japanese Patients with Eosinophilic Granulomatosis with Polyangiitis.
机构信息
From the Department of Allergy and Clinical Immunology, Chiba University Hospital; Department of Internal Medicine, Narita Red Cross Hospital; Department of Internal Medicine, Chiba Aoba Municipal Hospital; Department of Allergy and Clinical Immunology, Asahi General Hospital, Chiba; Department of Rheumatology, Dokkyo Medical University, Tochigi; Department of Rheumatology, Allergy and Clinical Immunology, National Hospital Organization Chiba-East Hospital, Chiba; Third Department of Internal Medicine, University of Yamanashi, Yamanashi; Department of Rheumatology, National Hospital Organization Shimoshizu Hospital; Department of Internal Medicine, Matsudo City Hospital; Centre for Rheumatic Diseases, Chibaken Saiseikai Narashino Hospital, Chiba, Japan.
A. Saku, MD, Chiba University Hospital; S. Furuta, MD, PhD, Chiba University Hospital; M. Hiraguri, MD, PhD, Narita Red Cross Hospital; K. Ikeda, MD, PhD, Chiba University Hospital; Y. Kobayashi, MD, PhD, Chiba Aoba Municipal Hospital; S.I. Kagami, MD, PhD, Asahi General Hospital; K. Kurasawa, MD, PhD, Dokkyo Medical University; R. Matsumura, MD, PhD, Chiba-East Hospital; D. Nakagomi, MD, PhD, University of Yamanashi; T. Sugiyama, MD, PhD, Shimoshizu Hospital; T. Umibe, MD, PhD, Matsudo City Hospital; N. Watanabe, MD, PhD, Chibaken Saiseikai Narashino Hospital; H. Nakajima, MD, PhD, Chiba University Hospital.
出版信息
J Rheumatol. 2018 Aug;45(8):1159-1166. doi: 10.3899/jrheum.171352. Epub 2018 Jun 15.
OBJECTIVE
Patients with eosinophilic granulomatosis with polyangiitis (EGPA) frequently experience relapses, which lead to cumulative organ damage. In this retrospective observational study, we aimed to reveal the risk factors for relapse in EGPA.
METHODS
A total of 188 Japanese patients with EGPA diagnosed between 1996 and 2015 were identified from medical records in 10 hospitals. The diagnosis was based on the American College of Rheumatology 1990 criteria or Lanham's criteria. Baseline characteristics, treatments, asthma exacerbation, and relapses were evaluated by retrospective chart review.
RESULTS
The median followup period was 56 months. The median age at disease onset was 59.7 years. At the disease onset, 95.2% of the patients had a history of bronchial asthma and 44.7% were positive for antineutrophil cytoplasmic antibodies. The cumulative survival and relapse-free survival rates at 5 years were 89.6% and 64.0%, respectively. Multivariate analysis with 2 models, proportional hazards, and competing risk models, was performed to identify the factors associated with relapse. The proportional hazards model identified azathioprine (AZA) maintenance therapy and high eosinophil counts at onset as independent factors with lower relapse risks, and high immunoglobulin E (IgE) levels at onset as a risk factor for relapse. The competing risk model identified no statistically significant factors.
CONCLUSION
Although potential benefit of AZA maintenance therapy in preventing relapse of EGPA was suggested by the proportional hazards model, there was a discrepancy in the results between the models. Eosinophil counts and IgE levels at onset were also identified as candidates of factors associated with relapse in EGPA.
目的
嗜酸性肉芽肿伴多血管炎（EGPA）患者常反复发作，导致累积性器官损伤。在这项回顾性观察研究中，我们旨在揭示 EGPA 复发的危险因素。
方法
从 10 家医院的病历中确定了 1996 年至 2015 年间诊断的 188 例日本 EGPA 患者。诊断标准基于美国风湿病学会 1990 年标准或 Lanham 标准。通过回顾性病历审查评估基线特征、治疗、哮喘加重和复发情况。
结果
中位随访时间为 56 个月。疾病发病的中位年龄为 59.7 岁。发病时，95.2%的患者有支气管哮喘病史，44.7%的患者抗中性粒细胞胞质抗体阳性。5 年累积生存率和无复发生存率分别为 89.6%和 64.0%。采用比例风险和竞争风险模型进行多变量分析，以确定与复发相关的因素。比例风险模型确定了硫唑嘌呤（AZA）维持治疗和发病时高嗜酸性粒细胞计数是复发风险较低的独立因素，而发病时高免疫球蛋白 E（IgE）水平是复发的危险因素。竞争风险模型未确定具有统计学意义的因素。
结论
虽然比例风险模型提示 AZA 维持治疗可能对预防 EGPA 复发有益，但模型之间存在差异。发病时的嗜酸性粒细胞计数和 IgE 水平也被认为是与 EGPA 复发相关的因素候选者。
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