莫須瘤於癌德星治療反應不佳之結締組織疾病合併間質性肺病者之療效

Effect of rituximab in patients with cyclophosphamide-non-responsive connective tissue disease-related interstitial lung disease: a case series study

目的：報告北部一家醫學中心在結締組織疾病合併間質性肺病的患者以靜脈注射脈衝癌德星（cyclophosphamide）的臨床經驗及莫須瘤（rituximab）於癌德星治療反應不佳之間質性肺病者之療效。方法：本研究中，我們藉由病例回顧方式，收集曾經接受癌德星或是癌德星加上莫須瘤治療的間質性肺疾病的患者。所有的病人皆有接受癌德星治療而對於癌德星反應不佳者則接受莫須瘤治療。病人在治療前皆有接受肺功能評估，並於治療後第六個月再次評估肺功能。所有病人治療前皆有接受肺部高分辨率電腦斷層掃描（high resolution computed tomography）檢查。結果：本研究總共收集了10個病人。在接受治療後，有一定比例的病人肺功能有明顯的改善。癌德星治療後肺功能改善及癌德星加上莫須瘤治療後肺功能改善的比例分別為50%（5/10）、60%（3/5）。結論：結締組織疾病合併間質性肺病的患者接受癌德星治療有一定比例的病人肺功能會進步，而對癌德星治療反應不佳者加上莫須瘤的治療仍有一部分的病人肺功能可以獲得改善。在結締組織疾病合併間質性肺病的患者，可以將莫須瘤當作救援性治療，對於頑劣性間質性肺病的患者可以考慮及早接受癌德星及莫須瘤的合併治療。

Objective: To report our experience of intravenous cyclophosphamide pulse therapy and the effect of rituximab (RTX) therapy in patients with cyclophosphamide-non-responsive connective tissue disease associated interstitial lung disease (CTD-ILD). Methods: We retrospectively evaluated the medical records of patients with CTD-ILD. All patients received intravenous cyclophosphamide pulse (IVCYC) therapy. The patients who received IVCYC+RTX were non-responsive to IVCYC treatment. At 0 to 6 months prior to the treatment, these patients underwent a pulmonary function test (PFT) . High resolution computed tomography was performed before the initiation of treatment. Results: Ten patients were enrolled in this study. A significant percentage of patients showed stabilization or improvement of pulmonary function after treatment. The response rates of the IVCYC and IVCYC+RTX groups were 50% (5/10) and 60% (3/5), respectively. Conclusions: Patients with CTD-ILD who received IVCYC with or without RTX therapy showed a significant improvement in lung function. RTX therapy could be employed as a salvage therapy and combined with IVCYC early for refractory CTD-ILD.