Clark Kristina En
UCL Centre for Rheumatology, University College London, London, UK.
J Scleroderma Relat Disord. 2023 Feb;8(1):7-13. doi: 10.1177/23971983221126850. Epub 2022 Sep 30.
Both antineutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis are rare autoimmune diseases. Both have the potential for significant multi-organ involvement, and both carry high morbidity and mortality. Disease-specific autoantibodies in these conditions allow for risk stratification for organ-based complications, and for personalised therapeutic strategies. The concomitant presentation of antineutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis is rare, and only reported in up to 1.3% of systemic sclerosis cases. These patients present more frequently with anti-myeloperoxidase and anti-topoisomerase antibody profiles, with increased incidence of interstitial lung disease and renal involvement than would be expected in either disease independently. Appreciating the role of the autoantibodies in each disease state, and where they overlap, allows for the potential of a more personalised approach to managing these complex patients.
抗中性粒细胞胞浆抗体相关性血管炎和系统性硬化症均为罕见的自身免疫性疾病。两者都有可能累及多个重要器官,且发病率和死亡率都很高。这些疾病中的疾病特异性自身抗体有助于对基于器官的并发症进行风险分层,并制定个性化治疗策略。抗中性粒细胞胞浆抗体相关性血管炎和系统性硬化症同时出现的情况很少见,在系统性硬化症病例中报告的比例最高仅为1.3%。这些患者更常出现抗髓过氧化物酶和抗拓扑异构酶抗体谱,与单独的任何一种疾病相比,间质性肺病和肾脏受累的发生率更高。了解自身抗体在每种疾病状态中的作用以及它们的重叠之处,有助于为管理这些复杂患者采用更个性化的方法。