Journal of Clinical Immunology ( IF 7.2 ) Pub Date : 2023-09-27 , DOI: 10.1007/s10875-023-01587-4
Giulio Tessarin 1, 2 , Manuela Baronio 1 , Luisa Gazzurelli 1 , Stefano Rossi 1 , Marco Chiarini 3 , Daniele Moratto 3 , Silvia Clara Giliani 2 , Maria Pia Bondioni 4 , Raffaele Badolato 1 , Vassilios Lougaris 1
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Granulomatous lymphocytic interstitial lung disease (GLILD) represents a fatal immune dysregulatory complication in common variable immunodeficiency (CVID). Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategies are heterogeneous and essentially based on experts’ opinions and data from small case series or case reports.
We aimed to evaluate the efficacy and safety of first-line Rituximab monotherapy for CVID-related GLILD, by assessing symptoms and quality of life alterations, immunological parameters, pulmonary function tests, and lung computed tomography.
All six GLILD patients received Rituximab infusions as a first-line treatment. Rituximab was administered at 375 mg/m2 monthly for six infusions followed by maintenance every 3 months; none of the patients experienced severe adverse events. Symptom burden and quality of life significantly improved in treated patients compared to a control group of CVID patients without GLILD. Rituximab treatment indirectly caused a trend toward reduced T-cell activation and exhaustion markers sCD25 and sTIM-3. Lung function improved in treated patients, with statistically significant increases in TLC and DLCO. Lung CT scan findings expressed by means of Baumann scoring system displayed a reduction in the entire cohort.
In conclusion, first-line monotherapy with Rituximab displayed high efficacy in disease remission in all treated patients, with improvement of symptoms and amelioration of quality of life, as well as restoration of PFTs and lung CT scan findings.
中文翻译:
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利妥昔单抗单药疗法作为 CVID 患者肉芽肿性淋巴细胞间质性肺病 (GLILD) 的一线治疗有效
肉芽肿性淋巴细胞间质性肺病 (GLILD) 是常见变异型免疫缺陷 (CVID) 中一种致命的免疫失调并发症。缺乏循证诊断指南,GLILD治疗以免疫抑制药物为主;然而,治疗策略是多种多样的,并且基本上基于专家的意见和小病例系列或病例报告的数据。
我们的目的是通过评估症状和生活质量改变、免疫学参数、肺功能测试和肺计算机断层扫描,评估一线利妥昔单抗单药治疗 CVID 相关 GLILD 的疗效和安全性。
所有六名 GLILD 患者均接受利妥昔单抗输注作为一线治疗。利妥昔单抗以 375 mg/m 2每月给药六次,随后每 3 个月维持一次;没有患者出现严重不良事件。与没有 GLILD 的 CVID 患者对照组相比,接受治疗的患者的症状负担和生活质量显着改善。利妥昔单抗治疗间接导致 T 细胞活化和耗竭标志物 sCD25 和 sTIM-3 减少的趋势。接受治疗的患者肺功能得到改善,TLC 和 DL CO显着增加,具有统计学意义。通过鲍曼评分系统表达的肺部 CT 扫描结果显示整个队列的情况有所减少。
总之,利妥昔单抗一线单药治疗对所有接受治疗的患者的疾病缓解均表现出高效,症状改善,生活质量改善,PFT 和肺部 CT 扫描结果恢复。