Cappelli Susanna, Bellando Randone Silvia, Camiciottoli Gianna, De Paulis Amato, Guiducci Serena, Matucci-Cerinic Marco
Dept of Experimental and Clinical Medicine, Division of Rheumatology AOUC, School of Medicine, University of Florence, Florence, Italy
Dept of Experimental and Clinical Medicine, Division of Rheumatology AOUC, School of Medicine, University of Florence, Florence, Italy.
Eur Respir Rev. 2015 Sep;24(137):411-9. doi: 10.1183/16000617.00002915.
Interstitial lung disease (ILD) is common in systemic sclerosis (SSc) patients and despite recent advances in the treatment is, at present, the major cause of death. Today, an early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease. Pulmonary function tests and high-resolution computed tomography remain the mainstay for the diagnosis of SSc-ILD, but there is a growing interest in lung ultrasound. Recently, the correlation between severity of fibrosis and some peripheral blood biomarkers has been described. Nonselective immunosuppressors are still the main treatment for ILD, with cyclophosphamide (CYC) most widely used to obtain remission. Novel therapies towards specific molecular and cellular targets have been suggested; in particular, rituximab (RTX) has shown promising results, but further research is needed. It is of paramount importance to define the severity of the disease and the risk of progression in order to define the need for treatment and the treatment intensity. We propose the division of the treatment strategies at our disposal to induce remission into three categories: high intensity (haematopoietic stem cell transplantation), medium intensity (CYC and RTX) and low intensity (azathioprine (AZA) and mycophenolate mofetil (MMF)). After obtaining remission, maintenance treatment with AZA or MMF should be started. In this review we explore new advances in the pathogenesis, diagnosis and treatment of SSc-ILD.
间质性肺病（ILD）在系统性硬化症（SSc）患者中很常见，尽管近年来治疗有了进展，但目前仍是主要死因。如今，ILD的早期诊断成为可能，这对于改善疾病预后至关重要。肺功能测试和高分辨率计算机断层扫描仍然是SSc-ILD诊断的主要手段，但肺超声正受到越来越多的关注。最近，已有人描述了纤维化严重程度与一些外周血生物标志物之间的相关性。非选择性免疫抑制剂仍然是ILD的主要治疗方法，其中环磷酰胺（CYC）应用最为广泛以实现缓解。有人提出了针对特定分子和细胞靶点的新疗法；特别是，利妥昔单抗（RTX）已显示出有前景的结果，但仍需要进一步研究。明确疾病的严重程度和进展风险对于确定治疗需求和治疗强度至关重要。我们建议将现有的诱导缓解的治疗策略分为三类：高强度（造血干细胞移植）、中等强度（CYC和RTX）和低强度（硫唑嘌呤（AZA）和霉酚酸酯（MMF））。在获得缓解后，应开始使用AZA或MMF进行维持治疗。在本综述中，我们探讨了SSc-ILD在发病机制、诊断和治疗方面的新进展。
