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混合性结缔组织病中的间质性肺疾病:一项高级检索

Interstitial Lung Disease in Mixed Connective Tissue Disease: An Advanced Search.

作者信息

Santacruz Juan Camilo, Mantilla Marta Juliana, Rodriguez-Salas Gustavo, Rueda Igor, Pulido Sandra, Varela Diana Cristina, Londono John

机构信息

Spondyloarthropathies Research Group, Universidad de La Sabana, Chía, COL.

Rheumatology Department, Universidad Militar Nueva Granada, Bogotá, COL.

出版信息

Cureus. 2023 Mar 15;15(3):e36204. doi: 10.7759/cureus.36204. eCollection 2023 Mar.

Abstract

The spectrum of pulmonary manifestations associated with mixed connective tissue disease ranges from pulmonary hypertension and interstitial lung disease to pleural effusions, alveolar hemorrhage, and complications from the thromboembolic disease. Interstitial lung disease in mixed connective tissue disease is a frequently occurring entity, although in most cases it tends to be self-limited or slowly progressive. Despite this, a significant percentage of patients may present a progressive fibrosing phenotype, thus posing a great challenge regarding its therapeutic approach, given the scarcity of clinical studies that compare the efficacy of immunosuppressants available to date. Due to this, many recommendations are extrapolated from other diseases with similar characteristics such as systemic sclerosis and systemic lupus erythematosus. That is why it is proposed to carry out an advanced search of the literature in order to clarify its clinical, radiological, and therapeutic characteristics to achieve its evaluation from a holistic point of view.

摘要

与混合性结缔组织病相关的肺部表现谱范围广泛,从肺动脉高压和间质性肺疾病到胸腔积液、肺泡出血以及血栓栓塞性疾病的并发症。混合性结缔组织病中的间质性肺疾病是一种常见病症,尽管在大多数情况下它往往是自限性的或缓慢进展的。尽管如此,相当一部分患者可能会呈现进行性纤维化表型,鉴于目前比较现有免疫抑制剂疗效的临床研究稀缺,这给其治疗方法带来了巨大挑战。因此,许多建议是从其他具有相似特征的疾病(如系统性硬化症和系统性红斑狼疮)中推断出来的。正因为如此,建议对文献进行深入检索,以阐明其临床、放射学和治疗特征,从而从整体角度对其进行评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8500/10103810/6e1e897c0e26/cureus-0015-00000036204-i01.jpg

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