《特发性肺纤维化诊断和治疗中国专家共识》发布

2016-07-11 2016年第6期《中华结核和呼吸杂志》 2016年第6期《中华结核和呼吸杂志》

2002年7月,中华医学会呼吸病学分会制定了《特发性肺(间质)纤维化诊断和治疗指南(草案)》。近十几年来,学界对特发性肺纤维化(IPF)的自然病程、病因学、发病机制和诊断方法都有了进一步认识,特别是抗肺纤维化药物的研发取得了一定进展,使IPF的治疗进入了新阶段。为规范IPF的诊断和治疗,中华医学会呼吸病学分会间质性肺疾病学组组织专家充分讨论后,结合国内外研究进展,参照国际最新指南,制订《特发性肺纤

2002年7月,中华医学会呼吸病学分会制定了《特发性肺(间质)纤维化诊断和治疗指南(草案)》。近十几年来,学界对特发性肺纤维化(IPF)的自然病程、病因学、发病机制和诊断方法都有了进一步认识,特别是抗肺纤维化药物的研发取得了一定进展,使IPF的治疗进入了新阶段。为规范IPF的诊断和治疗,中华医学会呼吸病学分会间质性肺疾病学组组织专家充分讨论后,结合国内外研究进展,参照国际最新指南,制订《特发性肺纤维化诊断和治疗中国专家共识》,供广大临床医师参考学习。

概念 IPF是一种病因不明、慢性进行性纤维化性间质性肺炎,病变局限在肺脏,好发于中老年男性人群,主要表现为进行性加重的呼吸困难,伴限制性通气功能障碍和气体交换障碍,导致低氧血症,甚至呼吸衰竭,预后差,其肺组织学和胸部高分辨率CT(HRCT)表现为普通型间质性肺炎(UIP)。

诊断标准 (1)排除其他已知原因的间质性肺疾病(如家庭或职业环境暴露、结缔组织病和药物毒性)。(2)HRCT表现为UIP型的患者,不建议行外科肺活检。(3)外科肺活检的患者,根据HRCT和外科肺活检特定的组合进行诊断。

治疗 (1)非药物治疗:包括戒烟、氧疗、机械通气、肺康复和肺移植。(2)药物治疗:酌情使用药物,目前尚无显著有效的治疗药物。(3)IPF急性加重的治疗:激素冲击或高剂量激素治疗。(4)姑息治疗:目的是减轻患者的症状,具体目标包括缓解躯体症状和减轻心理的焦虑和痛苦,给患者和家属精神上的支持。

IPF未来的治疗策略可能是针对肺纤维化不同靶点采用多种药物联合治疗。组织多中心的随机对照研究是评价IPF新的治疗药物的重要手段。

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2016 特发性肺纤维化诊断和治疗中国专家共识

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