Objective To explore the pathogenic features, clinical spectrum, risk factors, therapy and outcome in patients with connective tissue disease (CTD) complicated with invasive pulmonary fungal infections(IPFI). Methods A review was performed of 20 CTD patients with IPFI in our hospital between Jan 2006 and Jan 2010. Results The diagnostic status could be classified as follows:2 had confirmed diagnosis, 13 had clinical diagnosis,5 had suspected diagnosis. 17 patients had active disease. All patients with acute onset, fever (80%) common, may have varying degrees of respiratory symptoms . 10 patients had type I respiratory failure. 8 patients had leucopenia, 17patients had long- term use of corticosteroids, 10 patients received immunosuppressants and 12 patients received broad- spectrum antibiotics and 1 patient had invasive operation. The pathogen was Cryptococcus in 1 patient, Aspergillus in 1 patient, Candida albicans in 5 cases, Candida glabrata in 1 case , Candida krusei in 2 cases, Mucor in 1 case. And 9 patients had no pathogen data. Antifungal therapy was used in all patients. Of all cases, 16 survived and 4 died. Conclusion Clinicians should be alerted against CTD with IPFI, which is most frequently caused by Candida and usually highly fatal. Early diagnosis and rational treatment could improve the prognosis.%目的 探讨结缔组织病(CTD)合并侵袭性肺部真菌感染(IPFD的临床表现、病原学特征、易感因素、治疗和预后.方法 根据患者血、支气管灌洗及培真菌培养进行抗真菌治疗,并对其方法与转归进行回分析.结果 20例患者确诊2例,临床诊断13例,拟诊5例,17例患者有原发病活动.所有患者均急性起病,发热(80％)常见,可有不同程度的呼吸道症状.有10例患者并发急性Ⅰ型呼吸衰竭.合并有白细胞减少8例(40％),长期使用糖皮质激素治疗17例(85％),免疫抑制剂治疗10例(50％),使用广谱抗生素治疗12例(60％).侵袭性操作1例(5％).确诊曲霉菌及肺隐球菌各1例.查出白色念珠菌5例、光滑念珠菌1例及克柔念珠菌2例、毛霉菌1例,有9例患者无病原学资料.所有患者均接受抗真菌药,存活16例(80％),死亡4例(20％),其中3例患者合并有肺部间质纤维化、伴呼吸衰竭,1例患者系延迟抗真菌治疗.结论 CTD合并IPFI感染发生率高;起病急,病情重；临床症状不典型;可引起呼吸衰竭；病原菌检出机率不高,死亡率高,重视患者的宿主因素,积极的早期抗真菌治疗可有效改善预后.
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