肺部受累的特发性Sweet综合征三例并文献复习

Lung involvement of 3 cases of idiopathic sweet syndrome and literature review

目的 分析累及肺部的特发性Sweet综合征（SS）的临床特征.方法 回顾性分析广西医科大学第一附属医院2012年8月至2013年12月收治的3例肺部受累特发性SS患者临床资料,并复习文献.结果 以“pulmonary inflammation or involvement,lung”为关键词检索1981-2014年Pubmed数据库、清华同方和万方数据库,获取英文文献32篇36例,中文文献3篇3例,39例SS累及肺部的患者中31例合并有血液等系统疾病,8例未见其他合并症者诊断为特发性SS,将本组3例一并纳入分析,共收集特发性SS患者11例,其中男6例,女5例,平均年龄47岁（25 ～ 72岁）.均有发热、咳嗽、呼吸困难;痛性、红色假水疱样皮疹、肺部哕音、低氧血症及肺部渗出性阴影,淋巴结肿大（3例）.皮疹先于肺部受累6例,后于肺部受累5例.11例白细胞及中性粒细胞、ESR、CRP显著增高,肺部CT单侧或双侧肺部浸润性渗出、实变影,伴胸膜反应,限制性通气功能障碍（3/3）,BALF液（9/9）以中性粒细胞为主,肺组织病理（9/9）提示中性粒细胞浸润的间质性肺炎或伴机化性肺炎,与皮疹病理（11/11）一致.11例均误诊肺炎抗生素治疗无效,激素治愈9例,死亡2例.结论 特发性SS累及肺部临床罕见,可先于或后于皮疹出现,常误诊为细菌性肺炎.临床主要特征为不明原因的成熟中性粒细胞增多,发热、呼吸困难甚至呼吸衰竭、肺部渗出性或实变影,淋巴结肿大,红色、痛性假水疱样皮疹,受累部位大量中性粒细胞浸润.虽然激素有特效,但易复发,亦可导致死亡.

Objective To analyze the clinical features of idiopathic sweet＇ s syndrome （SS） that involves the lung.Methods A retrospective analysis was carried out on clinical data of 3 cases of patients with idiopathic SS that involves the lung who were admitted in the First Affiliated Hospital of Guangxi Medical University from August 2012 to December 2013.And relevant literatures were reviewed.Results Among the literatures reported between 1981 and 2014,there were a total of 39 SS cases involving the lung from both home and abroad,where 31 cases were accompanied with other diseases （i.e.blood diseases）;the rest 8 cases without comorbidities were diagnosed as idiopathic SS,which were included into the analysis plus the 3 cases of this group.Among 11 cases,6 cases were male while 5 cases were female,with the average age of 47 years old （25-72 years old）.The patients all had the symptoms of fever,cough and dyspnea;painful and red pseudo-blisters rashes,pulmonary rale,hypoxemia and pulmonary exudative shadow.3 cases had swollen lymph nodes.Rashes appeared before lung involvement in 6 cases while after lung involvement in 5 cases.In 11 cases,white blood cells,neutrophils,ESR and CRP were all significantly increased.Pulmonary CT showed unilateral or bilateral pulmonary invasive exudates and consolidation with pleural reaction.3 cases showed restrictive ventilatory dysfunction.BAF fluid （9/9cases） was given priority to neutrophils.Pulmonary pathology （9/9 cases） showed neutrophils-infiltrating interstitial pneumonia or organizing pneumonia,which were in accordance with the rash pathology （11/11cases）.11 cases all had been misdiagnosed as invalid antibiotic treatment on pneumonia;where 9 cases were healed by glucocorticoid while 2 cases died.Conclusions Idiopathic SS involving the lung is rare in clinical,which can appear before or after rash.Idiopathic SS is often misdiagnosed as bacterial pneumonia.Clinical features include the unknown increase of mature neutrophils,fever,dyspnea,and even respiratory failure,lung exudative or consolidation shadows,swollen lymph nodes and red and painful pseudo-blisters rashes.The involved area is often infiltrated by numerous neutrophils.The glucocorticoid has special effects,but easy to relapse,can also cause death.