Ikeda Takaharu, Komatsu Toshiro, Yokoyama Kae, Takahashi Kazuo, Kawakami Tamihiro
Division of Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.
J Dermatol. 2021 Apr;48(4):529-532. doi: 10.1111/1346-8138.15709. Epub 2020 Dec 10.
Treatment of eosinophilic granulomatosis with polyangiitis (EGPA) remains a challenge because currently available therapies, corticosteroids and immunomodulators, do not always control symptoms and are often associated with significant morbidity and relapse. Mepolizumab has been demonstrated to be an effective add-on therapy with steroid-sparing effect in cases of relapsing or refractory EGPA. Intravenous immunoglobulin (IVIG) therapy is effective against mononeuritis multiplex or heart failure in patients with EGPA who do not respond to corticosteroid-cyclophosphamide treatment. We present two cases of EGPA in which earlier add-on administration of adjunct mepolizumab and IVIG led to significant improvement in EGPA symptoms and prevention of flare-up of the disease. We suggested that earlier add-on combination administration of IVIG and mepolizumab might be a useful adjunct treatment to induce clinical remission of EGPA and improve the rate of remission, decrease relapse rate, and allow for reduced glucocorticoid use without any serious adverse drug effects.
嗜酸性肉芽肿性多血管炎（EGPA）的治疗仍然是一项挑战，因为目前可用的疗法，即皮质类固醇和免疫调节剂，并不总能控制症状，且常常伴有显著的发病率和复发。美泊利单抗已被证明是一种有效的附加疗法，在复发或难治性EGPA病例中具有激素节省作用。静脉注射免疫球蛋白（IVIG）疗法对那些对皮质类固醇 - 环磷酰胺治疗无反应的EGPA患者的多发性单神经炎或心力衰竭有效。我们报告了两例EGPA病例，其中早期附加使用美泊利单抗和IVIG导致EGPA症状显著改善并预防了疾病复发。我们认为，早期附加联合使用IVIG和美泊利单抗可能是一种有用的辅助治疗方法，可诱导EGPA临床缓解、提高缓解率、降低复发率，并允许减少糖皮质激素的使用，且无任何严重药物不良反应。
