风湿性疾病的肺部表现。
Pulmonary manifestations of rheumatologic diseases.
作者信息
Cidon Michal, Bansal Manvi, Hartl Dominik
机构信息
aDivision of Pediatric Rheumatology, Department of Pediatrics, USC Keck School of Medicine, Children's Hospital of Los Angeles, Los Angeles, California, USA bDivision of Pediatric Pulmonology, Department of Pediatrics, USC Keck School of Medicine, Children's Hospital of Los Angeles, Los Angeles, California, USA cDepartment of Pediatrics, University of Tübingen, Tübingen, Germany dRoche Pharma Research & Early Development (pRED), Immunology, Inflammation and Infectious Diseases (I3) Discovery and Translational Area, Roche Innovation Center, Basel, Switzerland.
出版信息
Curr Opin Pediatr. 2017 Jun;29(3):311-319. doi: 10.1097/MOP.0000000000000496.
PURPOSE OF REVIEW
The present review intends to provide an overview of the diversity and complexity of pulmonary manifestations of rheumatologic diseases and gaps in knowledge to effectively manage them.
RECENT FINDINGS
Diffuse lung disease in children with rheumatologic diseases represents a heterogeneous group of autoimmune disorders. Despite their significant morbidity and mortality, we have limited understanding about their pathogenesis. Here, we provide an overview of the pathophysiology and current management approach of these disorders, highlighting tools which assist with diagnosis, risk stratification and therapy. In this context, we address the need to develop a standardized approach to diagnose at-risk patients with rheumatologic disease and to predict their progression and the need to develop robust studies which evaluate the factors and interventions that influence pulmonary disease outcome.
SUMMARY
Diffuse lung disease in children with rheumatologic diseases represents a heterogeneous group of severe autoimmune disorders. By adopting a collaborative research approach among multicenters to help diagnose, risk stratify, and understand disease progression, effective management decisions can be optimized to improve clinical outcome.
综述目的
本综述旨在概述风湿性疾病肺部表现的多样性和复杂性，以及有效管理这些表现方面的知识空白。
最新发现
患有风湿性疾病的儿童弥漫性肺病是一组异质性自身免疫性疾病。尽管其发病率和死亡率很高，但我们对其发病机制的了解有限。在此，我们概述这些疾病的病理生理学和当前管理方法，重点介绍有助于诊断、风险分层和治疗的工具。在此背景下，我们阐述了制定标准化方法以诊断风湿性疾病高危患者并预测其病情进展的必要性，以及开展有力研究以评估影响肺部疾病转归的因素和干预措施的必要性。
总结
患有风湿性疾病的儿童弥漫性肺病是一组异质性严重自身免疫性疾病。通过多中心采用协作研究方法来帮助诊断、风险分层和了解疾病进展，可以优化有效的管理决策以改善临床结局。
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