■硬皮病,也被称为系统性硬化症,是一种多方面的自身免疫性疾病,其特征是异常纤维化和血管功能受损。病理上,它包括持续存在的炎症,胶原蛋白异常积聚,以及各种器官血管的重组,导致广泛的临床症状。这篇综述结合了关于硬皮病的最新科学文献,特别强调其病理生理学,临床表现,诊断方法,和治疗选择。
■对众多数据库进行了全面调查,比如PubMed,MEDLINE,Scopus,WebofScience,和谷歌学者,收集涵盖硬皮病研究各个方面的相关研究。
■硬皮病表现为一系列系统性表现,比如间质性肺病,胃肠动力障碍,雷诺现象,肺动脉高压,肾脏并发症,神经症状,和心脏异常。血清学标记,比如抗核抗体,抗着丝粒抗体,和抗拓扑异构酶抗体,对疾病分类和预测其结果很重要。
■硬皮病的准确识别对于及时正确实施有效的治疗计划至关重要。治疗方法旨在改善症状,减少并发症,并减缓疾病的进展。一种结合药理学药物的综合方法,包括免疫抑制剂,内皮素受体拮抗剂,和前列腺素,非药物干预措施,如物理和职业治疗是最大限度地提高病人护理至关重要。
■通过澄清现有的知识差距和对新兴趋势的识别,我们的目标是提高诊断的准确性,提高治疗干预措施的有效性,最终提高硬皮病患者的整体生活质量。持续的合作和创造性研究对于推进该领域并实现改善的患者结果和新的治疗发现是必要的。
UNASSIGNED: Scleroderma, also referred to as systemic sclerosis, is a multifaceted autoimmune condition characterized by abnormal fibrosis and impaired vascular function. Pathologically, it encompasses the persistent presence of inflammation, abnormal collagen buildup, and restructuring of blood vessels in various organs, resulting in a wide range of clinical symptoms. This review incorporates the most recent scientific literature on scleroderma, with a particular emphasis on its pathophysiology, clinical manifestations, diagnostic approaches, and treatment options.
UNASSIGNED: A comprehensive investigation was carried out on numerous databases, such as PubMed, MEDLINE, Scopus, Web of Science, and Google Scholar, to collect pertinent studies covering diverse facets of scleroderma research.
UNASSIGNED: Scleroderma presents with a range of systemic manifestations, such as interstitial lung disease, gastrointestinal dysmotility, Raynaud\'s phenomenon, pulmonary arterial hypertension, renal complications, neurological symptoms, and cardiac abnormalities. Serological markers, such as antinuclear antibodies, anti-centromere antibodies, and anti-topoisomerase antibodies, are important for classifying diseases and predicting their outcomes.
UNASSIGNED: The precise identification of scleroderma is crucial for promptly and correctly implementing effective treatment plans. Treatment approaches aim to improve symptoms, reduce complications, and slow down the progression of the disease. An integrated approach that combines pharmacological agents, including immunosuppressants, endothelin receptor antagonists, and prostanoids, with nonpharmacological interventions such as physical and occupational therapy is essential for maximizing patient care.
UNASSIGNED: Through the clarification of existing gaps in knowledge and identification of emerging trends, our goal is to improve the accuracy of diagnosis, enhance the effectiveness of therapeutic interventions, and ultimately enhance the overall quality of life for individuals suffering from scleroderma. Ongoing cooperation and creative research are necessary to advance the field and achieve improved patient outcomes and new therapeutic discoveries.