Bobeica Carmen, Niculet Elena, Craescu Mihaela, Parapiru Elena-Laura, Musat Carmina Liana, Dinu Ciprian, Chiscop Iulia, Nechita Luiza, Debita Mihaela, Stefanescu Victorita, Stefanopol Ioana Anca, Nechifor Alexandru, Pelin Ana Maria, Balan Gabriela, Chirobocea Silvia, Vasile Claudiu Ionut, Tatu Alin Laurentiu
Department of Morphological and Functional Sciences, Faculty of Medicine and Pharmacy, "Dunărea de Jos" University, Galați, Romania.
Multidisciplinary Integrated Center of Dermatological Interface Research MIC-DIR (Centrul Integrat Multidisciplinar de Cercetare de Interfata Dermatologica - CIM-CID), 'Dunărea de Jos' University, Galați, Romania.
J Inflamm Res. 2022 Jun 9;15:3387-3394. doi: 10.2147/JIR.S361667. eCollection 2022.
CREST syndrome is a clinical entity associated with systemic sclerosis, which meets at least three of the five clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Three of these clinical features (Raynaud's phenomenon, sclerodactyly and esophageal dysmotility) are often present in classical subsets of SSc: limited and diffuse, and their presence in association does not define CREST syndrome. Calcinosis seems to be less common in SSc and its association with other clinical features is characteristic of CREST syndrome. Therefore, it can be appreciated that calcinosis is the key element of CREST syndrome.
This study included a number of 37 candidates with SSc, diagnosed with the help of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2013 criteria.
These three elements (calcinosis, Raynaud's phenomenon, esophageal dysmotility) were recorded both in the limited subset of SSc, but especially in the subset of diffuse SSc, contrary to the data in the literature.
We appreciate that CREST syndrome is a clinical entity that can overlap with both subsets of SSc. Given the divergent views of the authors on the classification of CREST syndrome, future studies may contribute to a reassessment of SSc classification.
CREST综合征是一种与系统性硬化症相关的临床实体,它具备以下五个临床特征中的至少三个:钙质沉着、雷诺现象、食管动力障碍、指端硬化和毛细血管扩张。其中三个临床特征(雷诺现象、指端硬化和食管动力障碍)在系统性硬化症的经典亚型(局限性和弥漫性)中常常出现,它们同时出现并不足以定义CREST综合征。钙质沉着在系统性硬化症中似乎较少见,其与其他临床特征的关联是CREST综合征的特点。因此,可以认为钙质沉着是CREST综合征的关键要素。
本研究纳入了37名系统性硬化症患者,这些患者依据美国风湿病学会(ACR)/欧洲抗风湿病联盟(EULAR)2013年标准进行诊断。
这三个要素(钙质沉着、雷诺现象、食管动力障碍)在系统性硬化症的局限性亚型中均有记录,但在弥漫性系统性硬化症亚型中更为常见,这与文献数据相反。
我们认识到CREST综合征是一种可能与系统性硬化症的两个亚型都存在重叠的临床实体。鉴于作者们对CREST综合征分类存在不同观点,未来的研究可能有助于对系统性硬化症的分类进行重新评估。