Campochiaro Corrado, Lazzaroni Maria Grazia, Bruni Cosimo, Zanatta Elisabetta, De Luca Giacomo, Matucci-Cerinic Marco
Unit of Immunology, Rheumatology, Allergy and Rare Disease, IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy. Via Olgettina 60, 20132, Milan, Italy.
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia, Brescia, Italy.
Ther Adv Musculoskelet Dis. 2022 Aug 22;14:1759720X221116408. doi: 10.1177/1759720X221116408. eCollection 2022.
The results of randomized controlled (RCT) and retrospective studies have expanded the armamentarium of drugs for systemic sclerosis (SSc) - interstitial lung disease (ILD) treatment. The correct positioning of these drugs is not yet clarified.
Systemic literature review (SLR) on rituximab (RTX), tocilizumab (TCZ), nintedanib and abatacept (ABT) for the treatment of SSc-ILD. The results of the SLR were used to create a dedicated survey.
The study was performed as a systematic review.
the SLR was performed using the following terms: "(systemic sclerosis OR scleroderma) AND (interstitial lung disease OR lung fibrosis OR pulmonary fibrosis) AND (rituximab OR tocilizumab OR abatacept OR nintedanib)". The results of the SLR were integrated in a survey including 8 domains. These were sent to all EUSTAR members and to the participants of the 2020 Scleroderma World Congress.
41 studies (34 on RTX, 5 on TCZ, 2 on ABT, and 1 on nintedanib) were identified. RCTs supported the use of TCZ and nintedanib, while retrospective studies supported the use of RTX for SSc-ILD. No clear data were obtained about ABT. The survey showed that RTX is the most available option (96%) whereas the most frequent reason for targeted therapy introduction is lung progression while on csDMARDs (86% RTX, 59% TCZ and 63% nintedanib). Combination therapy was the most frequently mentioned therapeutic scheme for nintedanib (75%) and RTX (63%). Physicians' perception of safety was similar for all drugs, while drug efficacy was the same for RTX and nintedanib, followed by TCZ (4.8 ± 2). The most frequently raised concerns pertained to efficacy, safety and combination regimens.
Our SLR supports the use of RTX, TCZ and nintedanib for SSc-ILD patients and underlines the need for more data about upfront combination versus monotherapy. It also highlighted the need to identify predictors supporting drug choice according to both pulmonary and extra-pulmonary manifestations.
随机对照试验（RCT）和回顾性研究的结果扩大了系统性硬化症（SSc）-间质性肺疾病（ILD）治疗药物的种类。这些药物的正确定位尚未明确。
对利妥昔单抗（RTX）、托珠单抗（TCZ）、尼达尼布和阿巴西普（ABT）治疗SSc-ILD进行系统文献综述（SLR）。SLR的结果用于开展一项专门调查。
该研究作为系统评价进行。
使用以下检索词进行SLR：“（系统性硬化症或硬皮病）AND（间质性肺疾病或肺纤维化或肺间质纤维化）AND（利妥昔单抗或托珠单抗或阿巴西普或尼达尼布）”。SLR的结果整合到一项包含8个领域的调查中。这些调查被发送给所有EUSTAR成员以及2020年硬皮病世界大会的参会者。
共识别出41项研究（34项关于RTX，5项关于TCZ，2项关于ABT，1项关于尼达尼布）。RCT支持使用TCZ和尼达尼布，而回顾性研究支持RTX用于SSc-ILD。未获得关于ABT的明确数据。调查显示RTX是最容易获得的选择（96%），而引入靶向治疗最常见的原因是在使用传统合成改善病情抗风湿药（csDMARDs）时出现肺部进展（RTX为86%，TCZ为59%，尼达尼布为63%）。联合治疗是尼达尼布（75%）和RTX（63%）最常被提及的治疗方案。医生对所有药物安全性的认知相似，而RTX和尼达尼布的药物疗效相同，其次是TCZ（4.8±2）。最常提出的担忧涉及疗效、安全性和联合治疗方案。
我们的SLR支持RTX、TCZ和尼达尼布用于SSc-ILD患者，并强调需要更多关于初始联合治疗与单药治疗的数据。它还强调需要根据肺部和肺外表现确定支持药物选择的预测因素。
