Manfredi Andreina, Sebastiani Marco, Campomori Federica, Pipitone Nicolò, Giuggioli Dilia, Colaci Michele, Praino Emanuela, Ferri Clodoveo
From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena.
J Rheumatol. 2016 Aug;43(8):1575-80. doi: 10.3899/jrheum.160122. Epub 2016 Jun 15.
The term scleroderma pattern typically defines capillary abnormalities of scleroderma spectrum disorders, mainly systemic sclerosis (SSc) and dermatomyositis (DM). Our study aimed to investigate differences in nailfold videocapillaroscopy (NVC) between DM and SSc, with a cross-sectional and longitudinal evaluation.
NVC features of 29 consecutive patients with DM were compared with 90 patients with SSc categorized into the 3 subsets of scleroderma pattern: early, active, and late. Twenty patients with DM and all with SSc were also longitudinally reevaluated after 30 months of followup.
At baseline, all SSc groups showed giant capillaries, with significant differences with DM only for early and active pattern. Ramified capillaries were significantly more frequent and severe in DM than in early and active patterns, while DM showed an opposite trend compared with late pattern. Capillary loss was lower in early pattern and higher in active and late, compared with DM. Finally, giant-ramified capillaries were almost exclusive of DM. During followup, NVC showed a different evolution in DM and SSc. In DM we recorded a reduction of giant capillaries, while ramified capillaries increased both in DM and in early and active SSc pattern. The number of capillaries recovered in DM; conversely, capillary loss slightly worsened in all SSc patterns. Giant-ramified capillaries significantly decreased in patients with DM, remaining rare in patients with SSc.
Our study strengthens the specificity of DM and SSc microangiopathy and points out the need for large prospective studies to confirm our results and possibly to revise current terminology by distinguishing between "scleroderma" and "dermatomyositis" patterns.
硬皮病样模式一词通常定义硬皮病谱系疾病的毛细血管异常,主要是系统性硬化症(SSc)和皮肌炎(DM)。我们的研究旨在通过横断面和纵向评估,调查DM和SSc在甲襞视频毛细血管镜检查(NVC)方面的差异。
将29例连续的DM患者的NVC特征与90例SSc患者进行比较,后者被分为硬皮病样模式的3个亚组:早期、活动期和晚期。20例DM患者和所有SSc患者在随访30个月后也进行了纵向重新评估。
在基线时,所有SSc组均显示有巨大毛细血管,仅早期和活动期模式与DM有显著差异。分支毛细血管在DM中比早期和活动期模式更频繁且更严重,而与晚期模式相比,DM呈现相反趋势。与DM相比,早期模式的毛细血管丢失较少,活动期和晚期较高。最后,巨大分支毛细血管几乎仅见于DM。在随访期间,NVC显示DM和SSc有不同的演变。在DM中,我们记录到巨大毛细血管减少,而分支毛细血管在DM以及早期和活动期SSc模式中均增加。DM中的毛细血管数量恢复;相反,所有SSc模式中的毛细血管丢失略有恶化。DM患者的巨大分支毛细血管显著减少,在SSc患者中仍然罕见。
我们的研究强化了DM和SSc微血管病变的特异性,并指出需要进行大型前瞻性研究以证实我们的结果,并可能通过区分“硬皮病”和“皮肌炎”模式来修订当前术语。