Cassone Giulia, Sebastiani Marco, Vacchi Caterina, Erre Gian Luca, Salvarani Carlo, Manfredi Andreina
Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy.
Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
Drugs Context. 2021 Jan 15;10. doi: 10.7573/dic.2020-8-8. eCollection 2021.
Mycophenolate mofetil (MMF) is an antimetabolite with a potent inhibitory effect on proliferation of T and B lymphocytes used since the early 1990s for the prevention of acute allograft rejection after organ transplant. MMF is also widely used for the treatment of a variety of rheumatic diseases (RDs) and their pulmonary involvement. Interstitial lung disease (ILD) is a heterogeneous group of progressive fibrotic diseases of the lung, which is often secondary to RD and represents a major cause of morbidity and mortality. MMF is considered the main alternative to cyclophosphamide as a first-line agent to treat RD-related ILD or as possible maintenance therapy after cyclophosphamide, with a lower rate of side-effects. However, as for other immunosuppressive agents, the use of MMF in RD-ILD is supported by poor scientific evidence. In this narrative review, we describe the available data and recent advances on the effectiveness and safety of MMF for the treatment of ILD related to RD, including rheumatoid arthritis, systemic sclerosis, primary Sjögren syndrome, systemic lupus erythematosus, idiopathic inflammatory myopathies, undifferentiated connective tissue disease, interstitial pneumonia with autoimmune features and antineutrophil cytoplasmic antibody-associated vasculitis.
霉酚酸酯（MMF）是一种抗代谢物，对T和B淋巴细胞的增殖具有强大的抑制作用，自20世纪90年代初以来一直用于预防器官移植后的急性同种异体移植排斥反应。MMF还广泛用于治疗各种风湿性疾病（RDs）及其肺部受累情况。间质性肺疾病（ILD）是一组异质性的进行性肺纤维化疾病，通常继发于RD，是发病和死亡的主要原因。MMF被认为是环磷酰胺的主要替代药物，作为治疗RD相关ILD的一线药物或在环磷酰胺治疗后作为可能的维持治疗药物，副作用发生率较低。然而，与其他免疫抑制剂一样，MMF在RD-ILD中的应用缺乏充分的科学证据支持。在这篇叙述性综述中，我们描述了关于MMF治疗与RD相关的ILD（包括类风湿性关节炎、系统性硬化症、原发性干燥综合征、系统性红斑狼疮、特发性炎性肌病、未分化结缔组织病、具有自身免疫特征的间质性肺炎和抗中性粒细胞胞浆抗体相关血管炎）的有效性和安全性的现有数据及最新进展。
