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硬皮病的神经、心脏、肌肉骨骼和肾脏表现以及对其遗传学、病理生理学、诊断和治疗进展的见解。

Neurological, cardiac, musculoskeletal, and renal manifestations of scleroderma along with insights into its genetics, pathophysiology, diagnostic, and therapeutic updates.

作者信息

Prajjwal Priyadarshi, Marsool Mohammed Dheyaa Marsool, Yadav Vikas, Kanagala Ramya S D, Reddy Yeruva Bheemeswara, John Jobby, Lam Justin Riley, Karra Nanditha, Amiri Bita, Islam Moiz Ul, Nithya Venkatesh, Marsool Ali Dheyaa Marsool, Gadam Srikanth, Vora Neel, Hussin Omniat Amir

机构信息

Department of Neurology Bharati Vidyapeeth Deemed University Pune India.

Department of Neurology, Al-Kindy College of Medicine University of Baghdad Baghdad Iraq.

出版信息

Health Sci Rep. 2024 Apr 24;7(4):e2072. doi: 10.1002/hsr2.2072. eCollection 2024 Apr.

Abstract

BACKGROUND

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.

METHODOLOGY

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.

RESULTS

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.

DISCUSSION

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.

CONCLUSION

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.

摘要

背景

硬皮病,也称为系统性硬化症,是一种多方面的自身免疫性疾病,其特征为异常纤维化和血管功能受损。在病理上,它包括炎症的持续存在、异常的胶原蛋白积累以及各器官血管的重塑,从而导致广泛的临床症状。本综述纳入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。

方法

对多个数据库进行了全面调查,如PubMed、MEDLINE、Scopus、科学网和谷歌学术,以收集涵盖硬皮病研究各个方面的相关研究。

结果

硬皮病表现出一系列全身表现,如间质性肺疾病、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。血清学标志物,如抗核抗体、抗着丝点抗体和抗拓扑异构酶抗体,对于疾病分类和预测其预后很重要。

讨论

准确识别硬皮病对于及时、正确地实施有效的治疗方案至关重要。治疗方法旨在改善症状、减少并发症并减缓疾病进展。将包括免疫抑制剂、内皮素受体拮抗剂和前列腺素在内的药物与物理和职业治疗等非药物干预相结合的综合方法对于最大限度地提高患者护理水平至关重要。

结论

通过澄清现有知识差距并确定新出现的趋势,我们的目标是提高诊断准确性,增强治疗干预的有效性,并最终提高硬皮病患者的整体生活质量。持续的合作和创新性研究对于推动该领域发展、实现改善患者预后和新的治疗发现是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e500/11040569/1f328fef8203/HSR2-7-e2072-g001.jpg

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