Division of Pulmonary and Critical Care Medicine, Virginia Commonwealth University, Richmond, VA, USA.
Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, USA.
Lancet Respir Med. 2020 Mar;8(3):304-320. doi: 10.1016/S2213-2600(19)30480-1. Epub 2020 Feb 27.
Systemic sclerosis is an autoimmune connective tissue disease, which is characterised by immune dysregulation and progressive fibrosis that typically affects the skin, with variable internal organ involvement. It is a rare condition that affects mostly young and middle-aged women, resulting in disproportionate morbidity and mortality. Currently, interstitial lung disease is the most common cause of death among patients with systemic sclerosis, with a prevalence of up to 30% and a 10-year mortality of up to 40%. Interstitial lung disease is more common among African Americans and in people with the diffuse cutaneous form of systemic sclerosis or anti-topoisomerase 1 antibodies. Systemic sclerosis-associated interstitial lung disease most commonly presents with dyspnoea, cough, and a non-specific interstitial pneumonia pattern on CT scan, with a minority of cases fulfilling the criteria for usual interstitial pneumonia. The standard therapy has traditionally been combinations of immunosuppressants, particularly mycophenolate mofetil or cyclophosphamide. These immunosuppressants can be supplemented by targeted biological and antifibrotic therapies, whereas autologous haematopoietic stem-cell transplantation and lung transplantation are reserved for refractory cases.
系统性硬化症是一种自身免疫性结缔组织疾病，其特征为免疫失调和进行性纤维化，通常影响皮肤，伴有不同程度的内脏器官受累。这是一种罕见的疾病，主要影响年轻和中年妇女，导致不成比例的发病率和死亡率。目前，间质性肺疾病是系统性硬化症患者最常见的死亡原因，患病率高达 30%，10 年死亡率高达 40%。间质性肺疾病在非裔美国人和弥漫性皮肤型系统性硬化症或抗拓扑异构酶 1 抗体患者中更为常见。系统性硬化症相关的间质性肺疾病最常见的表现为呼吸困难、咳嗽和 CT 扫描上呈现非特异性间质性肺炎模式，少数病例符合寻常间质性肺炎的标准。传统的标准治疗是免疫抑制剂的联合治疗，特别是霉酚酸酯或环磷酰胺。这些免疫抑制剂可以通过靶向生物和抗纤维化治疗来补充，而自体造血干细胞移植和肺移植则保留用于难治性病例。
