Division of Rheumatology, Department of Medicine III, University Medical Centre and Faculty of Medicine Carl Gustav Carus at the TU Dresden, Germany.
Division of Respiratory Medicine, Department of Medicine I, University Medical Centre and Faculty of Medicine Carl Gustav Carus at the TU Dresden, Germany.
Clin Exp Rheumatol. 2022 Mar;40(3):483-488. doi: 10.55563/clinexprheumatol/o5t1f7. Epub 2021 Feb 25.
We aimed to analyse the effects of real-life immunomodulatory therapy with cyclophosphamide and rituximab for interstitial lung disease (ILD) in patients with systemic sclerosis (SSc-ILD), anti-synthetase syndrome (ASS-ILD), or Sjögren's syndrome (SjS-ILD), in a single academic centre.
All inpatients with connective tissue diseases treated with intravenous bolus cyclophosphamide or rituximab were identified from the Medical Centre records. Information on patient characteristics, chest CT results, pulmonary function tests, therapies, and severe adverse events, were extracted from inpatient and outpatient records.
Intravenous cyclophosphamide bolus therapy was used in 27 patients with SSc. Cyclophosphamide improved forced vital capacity (FVC) by more than 10% in 4 patients and stabilised it at -0.4% to +3.25% in 8. Rituximab constituted a rescue therapy in 14 SSc patients, and was used for treating 4 patients with ASS-ILD, 2 patients with SjS-ILD and one additional SSc-ILD patient. Rituximab led to FVC improvements of at least 5% in 8 patients and to stabilisation in another 6. 6 patients under cyclophosphamide and 8 patients under rituximab experienced severe adverse events. 8 of the 34 patients died, half of them from causes potentially related to therapy.
In this subset of severely sick patients with connective tissue diseases, cyclophosphamide and/or rituximab led to improvement in 12 patients, and stabilisation was seen in 14. Despite the new options with nintedanib, immunomodulation remains a relevant therapeutic modality for ILD associated with connective tissue disease.
我们旨在分析在单家学术中心，环磷酰胺和利妥昔单抗对系统性硬化症（SSc-ILD）、抗合成酶综合征（ASS-ILD）或干燥综合征（SjS-ILD）患者间质性肺病（ILD）的真实生活免疫调节治疗效果。
从医疗中心的记录中确定所有接受静脉注射环磷酰胺或利妥昔单抗治疗的结缔组织疾病住院患者。从住院和门诊记录中提取患者特征、胸部 CT 结果、肺功能检查、治疗和严重不良事件信息。
静脉注射环磷酰胺冲击治疗用于 27 例 SSc 患者。环磷酰胺使 4 例患者的用力肺活量（FVC）改善超过 10%，使 8 例患者的 FVC 稳定在-0.4%至+3.25%。利妥昔单抗构成 14 例 SSc 患者的挽救性治疗，用于治疗 4 例 ASS-ILD、2 例 SjS-ILD 和 1 例额外的 SSc-ILD 患者。利妥昔单抗使 8 例患者的 FVC 改善至少 5%，使另外 6 例患者的 FVC 稳定。环磷酰胺组 6 例患者和利妥昔单抗组 8 例患者发生严重不良事件。34 例患者中有 8 例死亡，其中一半患者的死亡与治疗潜在相关。
在这组患有严重结缔组织疾病的患者中，环磷酰胺和/或利妥昔单抗使 12 例患者的病情改善，使 14 例患者的病情稳定。尽管有尼达尼布等新选择，但免疫调节仍然是结缔组织疾病相关ILD 的一种相关治疗方式。
