Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary.
Department of Rheumatology, North Bristol NHS Trust, Bristol, UK; Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK.
Rheum Dis Clin North Am. 2023 May;49(2):211-248. doi: 10.1016/j.rdc.2023.01.002.
Systemic sclerosis (SSc) is a heterogenous systemic autoimmune disease of complex multi-organ manifestations with a disease-specific mortality of >50%. The patient journey is fraught with severe, diverse, and diffuse physical impairment, psychological burden, and diminishing health-related quality of life. SSc remains unfamiliar to many clinicians. Delayed/misdiagnosis, inadequate screening, and attention for common complications with potentially preventable disability/death contribute to patients feeling isolated and unsupported. We present actionable standards including screening, anticipatory guidance, and counseling in patient-centered SSc-care emphasizing psycho-social health as the central goal, whereas robust vigilance and efforts to improve biophysical health and survival are imperatives that support this goal.
系统性硬化症(SSc)是一种异质性全身性自身免疫性疾病,具有多种器官表现,其特定疾病死亡率>50%。患者的病程充满了严重、多样和弥漫性的身体损伤、心理负担以及健康相关生活质量的下降。许多临床医生对 SSc 并不熟悉。延迟/误诊、常规筛查不足以及对潜在可预防残疾/死亡的常见并发症的关注,导致患者感到孤立和无人支持。我们提出了一些可行的标准,包括在以患者为中心的 SSc 护理中进行筛查、预期指导和咨询,强调心理社会健康是核心目标,而强有力的警惕性和努力改善生物物理健康和生存则是支持这一目标的必要条件。