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目的探讨结缔组织病合并侵袭性肺曲霉菌病的易患因素、临床特征、治疗方法及预后。方法回顾性分析北京协和医院1995～2009年结缔组织病合并侵袭性肺曲霉菌病患者的临床资料。结果共检索肺曲霉菌病患者96例,合并结缔组织病患者20例。其中系统性红斑狼疮10例,抗中性粒细胞胞浆抗体(ANCA)相关性血管炎5例,炎性肌病5例。长期使用糖皮质激素14例,长期使用免疫抑制剂10例,长期使用广谱抗生素10例,低蛋白血症11例。临床症状无特异性。CT显示斑片浸润影12例,结节影10例,空洞形成4例。痰培养阳性19例,支气管灌洗液培养3例,纤支镜活检病理2例,经皮肺穿活检病理2例。初始治疗:给予二性霉素B7例,依曲康唑6例,伏立康唑7例。治愈5例,好转2例,未愈2例,死亡11例(55%)。结论结缔组织病合并侵袭性肺曲霉菌感染病死率髙,长期应用糖皮质激素、免疫抑制剂、广谱抗生素及低蛋白血症为结缔组织病并发侵袭性肺曲霉菌病的风险因素。及时进行反复痰培养、开展有创检查,如纤支镜下肺泡灌洗、肺活检有助早期诊断,改善预后。
Objective To investigate the predisposing factors, clinical features, treatment and prognosis of connective tissue disease complicated with invasive pulmonary aspergillosis. Methods The clinical data of patients with connective tissue disease complicated with invasive pulmonary aspergillosis in Peking Union Medical College Hospital from 1995 to 2009 were analyzed retrospectively. Results There were 96 patients with pulmonary aspergillosis and 20 patients with connective tissue disease. Including systemic lupus erythematosus in 10 cases, anti-neutrophil cytoplasmic antibody (ANCA) -related vasculitis in 5 cases, 5 cases of inflammatory myopathy. Long-term use of glucocorticoid in 14 cases, long-term use of immunosuppressive agents in 10 cases, long-term use of broad-spectrum antibiotics in 10 cases, hypoproteinemia in 11 cases. Clinical symptoms are not specific. CT showed patchy infiltration in 12 cases, 10 cases of nodules, empty formation in 4 cases. There were 19 cases of sputum culture positive, 3 cases of bronchoalveolar lavage fluid culture, 2 cases of biopsy by fiberoptic bronchoscopy and 2 cases of percutaneous pulmonary biopsy. Initial treatment: given amphotericin B 7 cases, itraconazole 6 cases, voriconazole 7 cases. 5 cases were cured, 2 cases were improved, 2 cases were unhealed and 11 cases died (55%). Conclusions The mortality rate of connective tissue disease complicated by invasive pulmonary aspergillosis is high, and long-term glucocorticoid, immunosuppressive agents, broad-spectrum antibiotics and hypoproteinemia are risk factors of connective tissue disease with invasive pulmonary aspergillosis. Timely repeated sputum culture, invasive detection, such as bronchoscopy bronchoalveolar lavage, lung biopsy can help early diagnosis and improve prognosis.