Liossis Stamatis-Nick C, Bounia Constantina A
Division of Rheumatology, Patras University Hospital, Patras, Greece.
Department of Internal Medicine, University of Patras Medical School, Patras, Greece.
Front Med (Lausanne). 2022 Jun 30;9:937561. doi: 10.3389/fmed.2022.937561. eCollection 2022.
Autoimmune rheumatic diseases may affect vital organs with lung involvement being severe and difficult to treat manifestation. Systemic sclerosis (SSc) commonly affects the lung in the form of interstitial lung disease (ILD). ILD may be also seen in patients with rheumatoid arthritis (RA), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), inflammatory myositis (IM), antisynthetase syndrome (AS), and the ANCA-associated vasculitides (AAV). Rituximab (RTX) is an anti-CD20 B lymphocyte depleting mAb, often administered in the treatment of autoimmune rheumatic diseases. Although RTX is an off-label treatment for CTD-ILD, there are numerous reports providing data that is effective in improving both pulmonary function tests (PFTs) and chest computed tomography findings consistent with ILD. There are retrospective uncontrolled studies that assess RTX as a treatment of ILD in autoimmune diseases. These studies, apart from one, do not include patients with AAV-ILD. In SSc-ILD, in particular, there are both controlled and uncontrolled studies displaying encouraging results following B cell depletion. In addition, a number of retrospective uncontrolled studies and fewer prospective studies evaluate RTX in connective tissue diseases CTD-ILD. Although RTX is an approved treatment for AAV there are scarce only data focusing on patients with AAV-ILD specifically. The results of a handful of studies comparing treatment of CTD-ILD with RTX to treatment with other agents are in favor of RTX. Results from large, still ongoing controlled trials are awaited to ascertain RTX effects in ILD encountered in autoimmune rheumatic diseases. We review herein the results of the different RTX trials in patients with autoimmune disease-associated with ILD. Despite the heterogeneity of these studies, RTX may be considered an alternative and safe but still off-label treatment for patients with refractory CTD-ILD.
自身免疫性风湿性疾病可能累及重要器官，肺部受累是严重且难以治疗的表现形式。系统性硬化症（SSc）常以间质性肺病（ILD）的形式累及肺部。类风湿关节炎（RA）、干燥综合征（SS）、系统性红斑狼疮（SLE）、炎性肌病（IM）、抗合成酶综合征（AS）以及抗中性粒细胞胞浆抗体相关性血管炎（AAV）患者也可能出现ILD。利妥昔单抗（RTX）是一种抗CD20 B淋巴细胞清除单克隆抗体，常用于治疗自身免疫性风湿性疾病。尽管RTX用于CTD-ILD的治疗属于超说明书用药，但有大量报告提供的数据表明，它在改善肺功能测试（PFTs）以及与ILD相符的胸部计算机断层扫描结果方面是有效的。有回顾性非对照研究评估了RTX作为自身免疫性疾病中ILD的一种治疗方法。除一项研究外，这些研究均未纳入AAV-ILD患者。特别是在SSc-ILD中，有对照和非对照研究均显示B细胞清除后结果令人鼓舞。此外，一些回顾性非对照研究和较少的前瞻性研究评估了RTX在结缔组织病CTD-ILD中的应用。尽管RTX是AAV的一种获批治疗药物，但专门针对AAV-ILD患者的数据却很少。少数比较RTX与其他药物治疗CTD-ILD的研究结果支持RTX。仍在进行的大型对照试验的结果有待确定RTX在自身免疫性风湿性疾病相关ILD中的作用。我们在此回顾了不同RTX试验在自身免疫性疾病合并ILD患者中的结果。尽管这些研究存在异质性，但对于难治性CTD-ILD患者，RTX可被视为一种替代且安全但仍为超说明书的治疗方法。
