Royal Free Hospital.
Royal Free Hospital and University College London, London, UK.
Curr Opin Rheumatol. 2022 Nov 1;34(6):302-310. doi: 10.1097/BOR.0000000000000904. Epub 2022 Aug 29.
One of the key clinical challenges of systemic sclerosis (SSc) is diversity in clinical presentation, organ involvement and disease progression. Antinuclear autoantibodies (ANA) are central to the diagnosis of SSc. ANA specificities associated with distinct clinical patterns of organ and skin involvement. Understanding of the molecular differences and pathogenesis of scleroderma has helped further inform clinical acumen. Here, we provide an update on ANA on clinical profiling, management and future direction of SSc.
There has been further development in delineating clinical patterns in ANA, genetic susceptibility and antigen triggers predisposing to ANA subtypes. Sub-group analysis of recent clinical trials shows differing treatment responses to novel therapeutics.
ANA subtyping is likely to be firmly embedded into future classification systems. Beyond informing current management and monitoring of scleroderma patients, ANA subsets have implication on future research and clinical trial design.
系统性硬化症(SSc)的主要临床挑战之一是临床表现、器官受累和疾病进展的多样性。抗核抗体(ANA)是 SSc 诊断的核心。ANA 特异性与不同的器官和皮肤受累的临床模式相关。对硬皮病分子差异和发病机制的了解有助于进一步提高临床敏锐度。在这里,我们提供了 ANA 在临床分析、管理和 SSc 未来方向的最新进展。
ANA、遗传易感性和抗原触发与 ANA 亚型之间的关系,在描绘 ANA 的临床模式方面取得了进一步的发展。最近临床试验的亚组分析显示,新型治疗药物的治疗反应不同。
ANA 亚型分类很可能会被纳入未来的分类系统。ANA 亚群不仅为当前硬皮病患者的管理和监测提供信息,而且对未来的研究和临床试验设计也有影响。