Musumeci Maria, Vadalà Gianluca, Russo Fabrizio, Pelacchi Federica, Lanotte Angela, Denaro Vincenzo
a 1 Campus Bio-Medico University of Rome, Department of Orthopaedic and Trauma Surgery , Via Alvaro del Portillo 200, 00128 Rome, Italy +39 06 2254111192 ; +39 06 225411638 ;
b 2 Cell Factory, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico , Milan, Italy.
Expert Opin Ther Targets. 2015;19(12):1677-87. doi: 10.1517/14728222.2015.1068758. Epub 2015 Aug 26.
Dupuytren's disease (DD) is a proliferative fibromatosis of the hand, which causes permanent flexion contracture of the digits and, ultimately, loss of function. The treatment of DD is complex and involves surgical and nonsurgical approaches, with the goal of removing the affected tissue. New biological targets are under investigation in order to develop innovative therapies.
The etiology of DD is still unknown. Several authors who focused their studies on the genetics of DD recognized an inherited autosomal dominant pattern. Actually, DD is a multifactorial and complex disease. Myofibroblasts are thought to play a crucial role in its pathogenesis, although their origin is not clear.
There is a general consensus that a better understanding of cellular and molecular mechanisms of DD will lead to the design of more specific and effective treatment alternatives. In this review, the authors hypothesize a new biological model for DD pathology, where myofibroblasts enhance the reservoir of the disease acting as if in a vicious cycle. This could help, ultimately, in identifying new therapeutic strategies to treat this common and disabling fibroproliferative disorder.
杜普伊特伦挛缩症(DD)是一种手部增殖性纤维瘤病,可导致手指永久性屈曲挛缩,最终导致功能丧失。DD的治疗较为复杂,包括手术和非手术方法,目的是切除受影响的组织。目前正在研究新的生物靶点,以开发创新疗法。
DD的病因仍不清楚。一些专注于DD遗传学研究的作者认识到一种常染色体显性遗传模式。实际上,DD是一种多因素的复杂疾病。肌成纤维细胞被认为在其发病机制中起关键作用,尽管其起源尚不清楚。
人们普遍认为,更好地理解DD的细胞和分子机制将有助于设计出更具体、更有效的治疗方案。在本综述中,作者提出了一种新的DD病理学生物学模型,其中肌成纤维细胞通过恶性循环增强了疾病的病灶。这最终可能有助于识别治疗这种常见且致残的纤维增殖性疾病的新治疗策略。