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-  2016 

以肺减缩综合征为首发表现的系统性红斑狼疮一例报告并文献复习

DOI: doi:10.7507/1671-6205.2016091

Keywords: 肺减缩综合征, 系统性红斑狼疮, 膈肌运动障碍

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Abstract:

目的探讨肺减缩综合征的临床表现、实验室检查及影像学特点、治疗方案以及发病机制。 方法报道我科2009年收治的1例以肺减缩综合征为首发表现的系统性红斑狼疮患者。并通过Pubmed及万方数据库检索1965年至2015年报道的肺减缩综合征有关文献,分析肺减缩综合征相关的临床资料。 结果本例患者为27岁男性,以反复憋气伴胸痛为主要症状;胸部影像学提示双肺体积缩小,双侧膈肌抬高,未见显著肺间质及实质受累;肺功能示限制性通气障碍;实验室检查示白细胞、血小板下降、补体降低,抗核抗体、抗双链DNA抗体等阳性。据此诊断系统性红斑狼疮、肺减缩综合征。予激素联合免疫抑制剂治疗,随访5年余,患者症状缓解,影像学及肺功能未见明显改善。文献复习共检索到肺减缩综合征病例118例,女100例,男18例,平均年龄(32.98±14.66)岁。主要临床表现包括呼吸困难、胸痛、端坐呼吸,体格检查可见气促、下肺呼吸音减低,影像学检查可见肺体积缩小、膈肌抬高、膈肌运动异常,肺功能表现为限制性通气障碍。主要治疗为激素联合免疫抑制剂,茶碱、β受体激动剂及利妥昔单抗亦可用于治疗。 结论对于系统性红斑狼疮等自身免疫性疾病患者,出现影像学无法解释的呼吸困难及胸痛,需警惕肺减缩综合征可能,尽快完善检查并积极治疗

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