Department of Dermatology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
New York Medical College School of Medicine, Valhalla, NY, USA.
Expert Rev Clin Immunol. 2022 May;18(5):461-483. doi: 10.1080/1744666X.2022.2063841. Epub 2022 May 4.
Morphea is a chronic autoimmune fibrosing condition of the skin and underlying tissue with the potential for significant disease-associated morbidity. While the exact etiology of morphea is not fully elucidated, many studies have explored the immunologic drivers of this disease.
Using PubMed, we performed a systematic review on morphea, with a focus on both the immune-mediated pathophysiology and treatment of this disease. Based on these findings, we review the literature surrounding what is understood about the role of the immune system in disease onset and course. Additionally, we discuss current treatments used in this disease as well as the potential role for more targeted therapies in the future.
Much work remains to fully elucidate each step in the immunologic march causing morphea. However, there is evidence to suggest that the early inflammatory stages of morphea may be driven predominantly by immunologic events in the Th1/Th17 pathway, while the Th2 pathway may be responsible for the fibrosis and damage observed later in the disease. Standard of care treatments currently continue to focus on therapeutics with broad immune modulating properties. Further work exploring the immunologic underpinnings of morphea will facilitate more targeted treatment approaches over time.
硬斑病是一种慢性自身免疫性纤维性皮肤病及皮下组织疾病,可能导致严重的疾病相关发病率。虽然硬斑病的确切病因尚未完全阐明,但许多研究已经探讨了这种疾病的免疫驱动因素。
我们使用 PubMed 对硬斑病进行了系统综述,重点关注该疾病的免疫介导病理生理学和治疗。基于这些发现,我们回顾了有关免疫系统在疾病发病和病程中的作用的文献。此外,我们还讨论了目前用于治疗这种疾病的方法,以及未来更有针对性治疗方法的潜在作用。
要完全阐明导致硬斑病的免疫级联反应的每一个步骤,仍有许多工作要做。然而,有证据表明,硬斑病的早期炎症阶段可能主要由 Th1/Th17 途径中的免疫事件驱动,而 Th2 途径可能负责疾病后期观察到的纤维化和损伤。目前的标准治疗方法仍然继续关注具有广泛免疫调节特性的治疗方法。随着时间的推移,进一步研究硬斑病的免疫学基础将有助于更有针对性的治疗方法。