a Division of Rheumatology, St. Joseph's Health Care, Department of Medicine , University of Western Ontario , Ontario , Canada.
b Rheumatologist, Connective Tissue Disease Fellow , University of Western Ontario , Ontario , Canada.
Expert Rev Clin Immunol. 2018 Jul;14(7):573-582. doi: 10.1080/1744666X.2018.1485490. Epub 2018 Jun 20.
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by vascular dysfunction, fibrosis, inflammation and autoantibodies. The pathophysiology of SSc is not completely understood, and many patients acquire organ or tissue damage despite advances in treatment. Current treatments target affected organs with modest improvements. Areas covered: This review evaluates several treatment strategies for SSc based on involved organs including skin, pulmonary, cardiac, renal, musculoskeletal, and gastrointestinal. Currently, pulmonary hypertension and interstitial lung disease are the primary causes of increased mortality. We will outline an approach to treatment of SSc based on disease manifestations and current evidence. Expert commentary: This complex disease is currently treated with therapies developed for similar indications such as for vascular manifestations of SSc using idiopathic pulmonary arterial hypertension treatments. Future directions in this field may include combination and maintenance therapy that is currently used in other autoimmune diseases, and tailoring these treatments according to the patients' phenotype. This will hopefully increase the efficacy of available treatments and decrease mortality from SSc.
系统性硬化症（SSc）是一种罕见的自身免疫性结缔组织疾病，其特征为血管功能障碍、纤维化、炎症和自身抗体。SSc 的病理生理学尚未完全阐明，尽管治疗取得了进展，但许多患者仍会出现器官或组织损伤。目前的治疗方法针对受影响的器官，仅能取得适度的改善。
本文基于皮肤、肺部、心脏、肾脏、肌肉骨骼和胃肠道等受累器官，评价了 SSc 的几种治疗策略。目前，肺动脉高压和间质性肺病是导致死亡率增加的主要原因。我们将根据疾病表现和现有证据概述 SSc 的治疗方法。
目前，针对 SSc 的血管表现等类似适应证，采用特发性肺动脉高压治疗来治疗这种复杂疾病。该领域的未来发展方向可能包括联合和维持治疗，这些治疗目前已用于其他自身免疫性疾病，并根据患者的表型定制这些治疗方法。这有望提高现有治疗方法的疗效，并降低 SSc 的死亡率。
