Seeliger Benjamin, Förster Martin, Happe Janett, Forberg Thomas, Moeser Anne, Neumann Thomas, Kroegel Claus
From the Department of Respiratory Medicine, Hannover Medical School, Hannover; Medical Clinic III, University Clinic Jena, Jena, Germany; Kantonsspital St. Gallen, St. Gallen, Switzerland.
B. Seeliger, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, and Department of Respiratory Medicine, Hannover Medical School; M. Förster, PhD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; J. Happe, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; T. Forberg, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; A. Moeser, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; T. Neumann, MD, Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, and Department of Rheumatology, Kantonsspital St. Gallen; C. Kroegel, MD, PhD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital. Dr. Neumann and Dr. Kroegel contributed equally to this manuscript.
J Rheumatol. 2017 Jun;44(6):806-814. doi: 10.3899/jrheum.160907. Epub 2017 Apr 15.
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by frequent relapses following induction therapy. Interferon-α (IFN-α) can reverse the underlying Th2-driven immune response and has successfully induced remission in previous reports. We undertook this study to investigate its efficacy and safety in patients with EGPA.
We conducted a retrospective monocentric cohort study including 30 patients (16 women) with active EGPA under IFN-α treatment. Primary endpoints were remission induction, occurrence of relapses, prednisolone (PSL) dosage at time of remission, and adverse events. Remission was defined by a Birmingham Vasculitis Activity Score (BVAS) of 0. Pulmonary function tests were recorded at baseline and at time of remission. Health-related quality of life was analyzed by questionnaire at baseline and following 12 months of treatment.
At baseline, the median BVAS was 6 (interquartile range 4-13.5) and remission or partial response was achieved in 25/30 patients. After initiation of IFN-α treatment, the median PSL dosages could be reduced from 17.5 mg/day at baseline to 5.5 mg/day at time of remission. Following remission, 17 relapses (5 major) in 16 patients were observed. Pulmonary function tests improved and the time of hospitalization decreased. Adverse events at initiation of treatment were common, but mostly transient. Severe adverse events occurred during treatment in 4 patients (autoimmune hepatitis, n = 1; drug-induced neuropathy, n = 3).
IFN-α treatment results in high rate of remission and maintenance in EGPA with significant reduction in oral corticosteroids, although reversible adverse events may occur. IFN-α represents an alternative therapeutic option in cases of refractory to standard treatment.
嗜酸性肉芽肿性多血管炎（EGPA）的特点是诱导治疗后频繁复发。干扰素-α（IFN-α）可逆转潜在的Th2驱动的免疫反应，并且在既往报告中已成功诱导缓解。我们开展了这项研究以调查其在EGPA患者中的疗效和安全性。
我们进行了一项回顾性单中心队列研究，纳入30例接受IFN-α治疗的活动性EGPA患者（16例女性）。主要终点为诱导缓解、复发的发生、缓解时的泼尼松龙（PSL）剂量以及不良事件。缓解定义为伯明翰血管炎活动评分（BVAS）为0。在基线和缓解时记录肺功能测试结果。通过问卷调查在基线和治疗12个月后分析健康相关生活质量。
基线时，BVAS中位数为6（四分位间距4 - 13.5），25/30例患者实现缓解或部分缓解。开始IFN-α治疗后，PSL中位数剂量可从基线时的17.5 mg/天降至缓解时的5.5 mg/天。缓解后，观察到16例患者出现17次复发（5次为主要复发）。肺功能测试改善，住院时间缩短。治疗开始时不良事件常见，但大多为短暂性。4例患者在治疗期间发生严重不良事件（自身免疫性肝炎，1例；药物性神经病变，3例）。
IFN-α治疗可使EGPA患者获得高缓解率和缓解维持率，口服糖皮质激素显著减少，尽管可能发生可逆性不良事件。IFN-α是标准治疗难治病例的一种替代治疗选择。
