Campochiaro Corrado, Suliman Yossra A, Giuggioli Dilia, Moinzadeh Pia, Alunno Alessia, Schoones Jan W, Baron Murray, Chung Lorinda, Ross Laura, Maltez Nancy, Alcacer-Pitarch Begonya, El-Aoufy Khadija, Allanore Yannick, Del Galdo Francesco, Denton Christopher P, Distler Oliver, Frech Tracy, Furst Daniel E, Khanna Dinesh, Krieg Thomas, Kuwana Masataka, Matucci-Cerinic Marco, Pope Janet, Hughes Michael
Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milano, Italy.
Assiut University Hospital, Assiut, Egypt.
J Scleroderma Relat Disord. 2025 May 15:23971983251339821. doi: 10.1177/23971983251339821.
Digital ulcers (DUs) stand out as one of the most prevalent and clinically meaningful manifestations of systemic sclerosis (SSc) and are associated with significant morbidity. While systemic (pharmacological) therapy is currently established as the 'standard of care', effective local ulcer management remains crucial for all cases of DUs. This is particularly true for patients who cannot tolerate systemic treatments or in the case of refractory SSc-DUs. On this background, there is a pressing demand for the formulation of evidence-based guidelines to assist clinicians and patients in navigating the local treatment options for DUs.
A steering committee of international experts was established by the World Scleorderma Foundation (WSF) Digital Ulcer (DU) ad hoc committee. Two systematic literature reviews on local non-surgical and surgical treatments for the management of SSc-DUs were performed to inform the development of local treatment recommendations for SSc-DUs. Consensus methodology was used to develop the final treatment recommendations.
Six overarching treatment principles and eight local treatment recommendations (five non-surgical and three surgical) were agreed upon for the management of SSc-DU. Among topical non-surgical options, botulin toxin can be conditionally recommended for refractory and/or severe DUs. Among surgical treatments, autologous adipose tissue grafting might be recommended for DU healing when combined with background systemic treatments.
These recommendations are specifically tailored to guide treatment decisions concerning both local and non-pharmacological approaches to managing SSc-related DUs. Our work has highlighted a notable quality gap in comparison to systemic treatments, underscoring the scarcity of high-quality studies concerning this topic.
指端溃疡(DUs)是系统性硬化症(SSc)最常见且具有临床意义的表现之一,与显著的发病率相关。虽然目前全身性(药物)治疗已成为“标准治疗方案”,但有效的局部溃疡管理对于所有指端溃疡病例仍然至关重要。对于无法耐受全身治疗的患者或难治性SSc-DU患者而言尤其如此。在此背景下,迫切需要制定循证指南,以协助临床医生和患者选择指端溃疡的局部治疗方案。
世界硬皮病基金会(WSF)指端溃疡(DU)特设委员会成立了一个国际专家指导委员会。针对SSc-DU的局部非手术和手术治疗进行了两项系统文献综述,以为SSc-DU局部治疗建议的制定提供依据。采用共识方法制定最终治疗建议。
就SSc-DU的管理达成了六项总体治疗原则和八项局部治疗建议(五项非手术和三项手术)。在局部非手术选择中,肉毒杆菌毒素可有条件地推荐用于难治性和/或严重指端溃疡。在手术治疗中,自体脂肪组织移植与背景全身治疗联合使用时,可能有助于指端溃疡愈合。
这些建议是专门为指导有关管理SSc相关指端溃疡的局部和非药物方法的治疗决策而制定的。与全身治疗相比,我们的工作凸显了明显的质量差距,强调了关于该主题的高质量研究的匮乏。