Kowal-Bielecka Otylia, Bielecki Marek, Kowal Krzysztof
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Białystok, Poland.
Pol Arch Med Wewn. 2013;123(1-2):51-8. doi: 10.20452/pamw.1593. Epub 2013 Jan 21.
Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by widespread vascular injury and progressive fibrosis of the skin and internal organs. The involvement of internal organs results in significant morbidity and mortality of SSc patients with cardiopulmonary involvement being the leading cause of SSc‑related deaths. The management of SSc patients remains a challenge because therapeutic options are rather limited and no therapy has definitively shown a disease‑modifying effect. A significant progress that has recently been made in the understanding of the SSc pathogenesis contributed to the introduction of new therapeutic options. Preliminary clinical studies have yielded promising results for mycophenolate mofetil, anti‑CD20 antibodies, and stem‑cell in the treatment of SSc. Multicenter cohort studies help understand the natural history of SSc, which leads to improvement in the care of SSc patients. The major objective of those studies is to establish the screening strategies for early diagnosis and, subsequently, to introduce appropriate management concerning specific organ involvement in SSc as well as to formulate specific treatment recommendations.
系统性硬化症（SSc）是一种多系统自身免疫性疾病，其特征为广泛的血管损伤以及皮肤和内脏器官的进行性纤维化。内脏器官受累导致SSc患者出现显著的发病率和死亡率，心肺受累是SSc相关死亡的主要原因。由于治疗选择相当有限且尚无疗法明确显示出改善病情的效果，SSc患者的管理仍然是一项挑战。最近在SSc发病机制的理解方面取得的重大进展促使了新治疗选择的引入。初步临床研究已在霉酚酸酯、抗CD20抗体和干细胞治疗SSc方面取得了有前景的结果。多中心队列研究有助于了解SSc的自然病程，从而改善对SSc患者的护理。这些研究的主要目标是建立早期诊断的筛查策略，随后针对SSc中特定器官受累引入适当的管理措施，并制定具体的治疗建议。
