組織纖維化 - 多學科復康 | Tissue Fibrosis (MRP)

人類纖維化疾病是世界範圍內的一個主要健康問題，因為受累的個體數量眾多，對纖維化過程的發病機制認識不全，其病因和臨床表現具有明顯的異質性，缺乏適當和充分驗證的生物標記物，最重要的是，現時缺乏有效的治療疾病的藥物。纖維化疾病包括廣泛的臨床實體，包括系統性纖維化疾病，如系統性硬化症（SSc）、硬皮病移植物與宿主病、腎原性系統性纖維化，以及許多器官特异性疾病，包括輻射誘導的纖維化和心、肺、肝和腎纖維化。儘管這些疾病的致病機制多種多樣，而且在某些情况下仍然難以捉摸，但它們有一個共同的特點，即受累器官中纖維組織的不受控制和進行性積聚，導致其功能障礙和最終衰竭。儘管導致纖維化疾病發展的病因機制及其臨床表現存在顯著的異質性，但許多研究表明，活化的肌成纖維細胞是最終導致正常組織被無功能的纖維化組織替代的共同細胞成分。主要由轉化生長因數-β（TGF-β）啟動的關鍵訊號級聯反應，也涉及許多細胞因數和刺激肌成纖維細胞促纖維化反應的訊號分子，提供了潜在的治療靶點。在這裡，我們簡要回顧了現時有關組織纖維化發展的分子機制的知識，並指出了一些最重要的挑戰，以研究纖維化疾病和發展有效的治療方法，這往往是致命的疾病組。應鼓勵進一步闡明纖維化過程的複雜致病機制的努力，以實現為這些嚴重、無法治療且往往致命的疾病開發有效療法的難以捉摸的目標。

_{如你有任何疾病, 請遵照閣下醫生的完整醫療方案; 而是否使用多學科復康方案前, 你必須咨詢主診醫生的意見, 如果閣下的主診醫生不建議您加入補充劑調理組合, 請你不要使用。如果你需要尋求其他醫生作第二咨詢, 閣下可聯絡我們線上<無邊界醫生>或你自己城市內的其他專業醫生的再診斷。}

Human fibrotic diseases constitute a major health problem worldwide owing to the large number of affected individuals, the incomplete knowledge of the fibrotic process pathogenesis, the marked heterogeneity in their etiology and clinical manifestations, the absence of appropriate and fully validated biomarkers, and, most importantly, the current void of effective disease-modifying therapeutic agents. The fibrotic disorders encompass a wide spectrum of clinical entities including systemic fibrotic diseases such as systemic sclerosis (SSc), sclerodermatous graft vs. host disease, and nephrogenic systemic fibrosis, as well as numerous organ-specific disorders including radiation-induced fibrosis and cardiac, pulmonary, liver, and kidney fibrosis. Although their causative mechanisms are quite diverse and in several instances have remained elusive, these diseases share the common feature of an uncontrolled and progressive accumulation of fibrotic tissue in affected organs causing their dysfunction and ultimate failure. Despite the remarkable heterogeneity in the etiologic mechanisms responsible for the development of fibrotic diseases and in their clinical manifestations, numerous studies have identified activated myofibroblasts as the common cellular element ultimately responsible for the replacement of normal tissues with nonfunctional fibrotic tissue. Critical signaling cascades, initiated primarily by transforming growth factor-β (TGF-β), but also involving numerous cytokines and signaling molecules which stimulate profibrotic reactions in myofibroblasts, offer potential therapeutic targets. Here, we briefly review the current knowledge of the molecular mechanisms involved in the development of tissue fibrosis and point out some of the most important challenges to research in the fibrotic diseases and to the development of effective therapeutic approaches for this often fatal group of disorders. Efforts to further clarify the complex pathogenetic mechanisms of the fibrotic process should be encouraged to attain the elusive goal of developing effective therapies for these serious, untreatable, and often fatal disorders.

_{Ref: Joel Rosenbloom 1 ^, Edward Macarak 1 ^, Sonsoles Piera-Velazquez 1 ^, Sergio A Jimenez 2 ^Human Fibrotic Diseases: Current Challenges in Fibrosis Research. Review. Methods Mol Biol. 2017;1627:1-23. doi: 10.1007/978-1-4939-7113-8_1.}

_{If you have a medical condition, please follow your doctor's medical treatment plan. Do consult your medical doctor before using the Multidisciplinary Rehabilitation Program (MRP). Please do not proceed if your medical doctor does not recommend the use of our MRP. If you'd like to seek a second opinion from other doctors, you may contact our online "Doctors Without Border", or other professional doctors at your convenience.}