异形性炎症性肌肉病相关的间歇性肺病的临床,功能和计算机断层扫描特征:一项回顾性队列研究
José Ricardo Bandeira de Oliveira Filho1, André Nathan Costa1, Bruno Guedes Baldi1
1. Divisão de Pneumologia, Instituto do Coração - InCor - Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo - HCFMUSP - São Paulo (SP) Brasil.
与异形性炎症性肌肉病相关的间歇性肺病 (IIM-ILD) 患者通常通过免疫抑制疗法得到改善. 然而,肺高血压和高剂量的类固醇可能会增加IIM-ILD的死亡风险.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 放射学 放射学是一门学科.
背景情况:
- 与异形性炎症性肌肉病相关的间歇性肺病 (IIM-ILD) 是一个重大的临床挑战.
- 了解IIM-ILD的特征和进展对于患者管理至关重要.
研究的目的:
- 评估IIM-ILD患者的临床,功能和CT特征.
- 评估IIM-ILD患者在巴西转诊中心接受治疗的疾病进展和结果.
主要方法:
- 79名IIM-ILD患者的回顾性队列研究.
- 分析包括人口统计,临床数据,肺功能测试,HRCT,血清学,治疗方法和结果.
- 平均随访时间为8.7年.
主要成果:
- 抗合成酶综合征是最常见的诊断 (64.5%).
- 呼吸不全 (94.9%),关节痛 (82.2%),肌肉虚弱 (75.9%) 是最常见的症状.
- 平均基线FVC预测为62.5%,随访期间有显著改善.
- 不确定的 (44.4%) 和非特异性的间歇性肺炎 (35.4%) 是常见的CT模式.
- 普雷尼松 (98.7%),阿扎西奥普林 (92.3%) 和甲状腺素 (57.7%) 是常见的治疗方法.
- 总体存活率为84.8%. 这是一个令人惊叹的数字.
结论:
- 大多数IIM-ILD患者经历了免疫抑制治疗的良好结果.
- 肺高血压和需要静脉注射甲基prednisolone脉冲治疗与死亡率的增加有关.
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