皮肌炎合并侵袭性肺部真菌感染(IPFI)经验性治疗的临床分析

 目的    分析皮肌炎合并侵袭性肺部真菌感染(invasive pulmonary fungal infections,IPFI)经验性治疗的疗效。方法    回顾性分析2011年11月至2017年6月复旦大学附属华山医院风湿免疫科收治的15例皮肌炎合并IPFI患者的临床资料,根据病史、临床表现及实验室检查,参照IPFI的诊断标准与治疗原则［1］,2例患者达到临床诊断标准,13例患者达到拟诊标准,所有患者给予经验性抗真菌治疗,依据临床疗效指标评价疗效。结果    15例患者均有活动性胸闷、气促,血气分析显示7例患者有Ⅰ型呼衰,8例患者有低氧血症,10例有单侧靠近胸膜的新发浸润病灶(高密度影或结节影,其中2例患者有空洞)。经过治疗15例患者出院前IPFI症状、体征、血气分析指标等明显改善,8例影像学病灶好转大于50%,4例影像学病灶好转小于50%。根据疗效评判标准,11例病例显效,4例患者进步,有效率为73%。结论    早期持续性低氧血症或Ⅰ型呼衰、新发单侧胸膜下高密度浸润病灶或结节影,特别是伴有双轨征或空洞是皮肌炎合并IPFI的早期识别依据;经验性抗真菌治疗是有效的治疗方法,能改善皮肌炎合并IPFI患者的预后。

Assessment of empirical treatment in dermatomyositis combined with invasive pulmonary fungal infections (IPFI)

Objective    To investigate the efficacy of early empirical therapy on dermatomyositis combined with invasive pulmonary fungal infections (IPFI).Methods    We retrospectively evaluated 15 patients with dermatomyositis accompanying with IPFI enrolled in Departmet of Rheumatology,Huashan Hospital,Fudan University between Nov.,2011 and Jun.,2017.According to the medical history,clinical manifestations and laboratory studies,2 patients met the standard for clinical diagnosis and 13 patients were considered as suspected diagnosis (Diagnostic Criteria and Principles of Treatment for IPFI［1］).All patients were given the empirical antifungal therapy.The effectiveness of the treatment were evaluated based on clinical features and laboratory studies.Results     Fifteen patients showed active chest tightness and shortness of breath,7 cases had type Ⅰ respiratory failure according to blood gas analysis,8 cases had hypoxemia,and 10 developed new infiltrating lesions near the unilateral pleura (high-density shadow or nodules,2 cases had cavity).After the treatment,significantly improvement in clinical presentations,blood gas indexes and imaging abnormalities prior discharge from hospital was observed in all 15 patients.Eight patients showed move than 50% improvement in lung lesions,while 4 cases with less than 50%.According to the curative effect evaluation criteria,11 cases were deemed as significantly effective,and 4 cases had mild improvement,which resulted in effective rate of 73%.Conclusions    Early persistent hypoxemia,type Ⅰ respiratory failure,new unilateral pleural high density infiltrating lesions or nodules,especially with double track sign or cavity are the early clinical features for IPFI in dermatomyositis.Empirical antifungal therapy is effective in treating dermatomyositis complicated with IPFI with improment of outcome.