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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。
Objective To investigate the clinical manifestations, laboratory tests and imaging features, treatment options and pathogenesis of pulmonary contracture syndrome. Methods reported in our department in 2009 admitted to a case of lung reduction syndrome as the first manifestation of systemic lupus erythematosus patients. PubMed and Wanfang database were used to search the related literature of lung shrinkage syndrome reported from 1965 to 2015 to analyze the clinical data related to lung shrinkage syndrome. Results The patient was 27-year-old with repeated breath-hold and chest pain as the main symptom. Thoracic imaging showed a reduction in both lung volume and bilateral diaphragmatic elevation with no significant pulmonary interstitial and parenchymal involvement. Pulmonary function showed restrictive ventilatory dysfunction ; Laboratory tests showed white blood cells, thrombocytopenia, decreased complement, antinuclear antibodies, anti-double-stranded DNA antibodies positive. According to the diagnosis of systemic lupus erythematosus, pulmonary contract syndrome. Hormones combined immunosuppressive therapy, follow-up more than 5 years, patients with symptoms, imaging and pulmonary function no significant improvement. Literature review retrieved a total of 118 cases of lung shrinkage syndrome, 100 females, 18 males, mean age (32.98 ± 14.66) years old. The main clinical manifestations include dyspnea, chest pain, sitting breathing, physical examination showed shortness of breath, decreased lung breath sounds, imaging examination showed reduced lung volume, diaphragm elevation, diaphragmatic motion abnormalities, pulmonary function showed restrictive ventilation disorders. The main treatment for hormone combination immunosuppressive agents, theophylline, β-agonists and rituximab can also be used for treatment. Conclusions For patients with autoimmune diseases such as systemic lupus erythematosus, radiographic unexplained dyspnea and chest pain may require vigilance on the possibility of pulmonary contract syndrome, and the examination and aggressive treatment should be completed as soon as possible.