系统性红斑狼疮合并弥漫性肺泡出血一例并文献复习

A case report and literature review of systemic lupus erythematosus with diffuse alveolar hemorrhage

目的分析系统性红斑狼疮(systemic lupus erythematosus,SLE)合并弥漫性肺泡出血(diffuse alveolar hemorrhage,DAH)的临床特点,观察血浆置换联合激素救治该类患者的临床疗效,以提高对罕见病例的诊疗水平,避免误诊及延误治疗。方法回顾性分析2015年8月12日郑州大学第一附属医院收治的1例SLE合并DAH患者的临床表现,观察血浆置换联合激素冲击治疗该患者的临床疗效,同时综合分析国内报道的101例SLE合并DAH患者的临床表现、治疗方案及其临床效果。结果本例20岁女性患者,临床表现主要为发热,痰中带血,面颊部红斑,伴阴道出血;实验室检查结果提示抗核抗体(antinuclear antibodies,ANA)1∶1000,抗双链DNA(anti double stranded DNA,dsDNA)抗体阳性,抗Smith抗体阳性;贫血,血红蛋白进行性下降;胸部CT示两肺野见多发条片状,斑点状,结节状密度增高影,边缘模糊。诊断考虑SLE合并DAH,给予血浆置换联合激素冲击,同时给予人免疫丙种球蛋白应用,患者咯血停止,面颊部红斑消失,贫血较前明显改善,目前口服糖皮质激素并规律减量。我国文献报道类似病例101例,具体描述患者临床表现的有83例,这些患者中ANA均为阳性,抗ds-DNA抗体阳性者58例,咯血67例,发热61例,肾脏受累71例,血红蛋白下降74例,呼吸困难80例,低氧血症70例。影像学均显示肺部浸润影。通过血浆置换、激素冲击,免疫抑制剂等药物的应用,10例行血浆置换,7例有效;29例给予丙种球蛋白,21例有效;74例接受了大剂量激素冲击,47例有效;21例使用了机械通气,8例有效;49例给予免疫抑制剂,36例有效;12例使用了支气管肺泡灌洗,10例有效。结论 DAH是SLE罕见且威胁生命的并发症,尽早明确诊断,及时血浆置换联合激素冲击等对症支持治疗,积极防治并发症,可有效改善患者的临床表现及其预后。

Objective To analyze the clinical characteristics of systemic lupus erythematosus （SI.E） with diffuse alveolar hemorrhage （DAH）, and to study the efficacy of plasmapheresis combined with hormone in SLE with DA in order to improve the level of diagnosis and treatment of rare cases, and avoid misdiagnosis and delayed treatment. Methods The clinical manifestations of 1 ease of SLE with DAH in our hospital were reported, and the clinical effect of plasma exchange combined with corticosteroid for treatment of the patients was observed at the same time. A comprehensive analysis was clone on DAH clinical manifestations, treatment and clinical effects of 101 Chinese SLE patients. Results This patient was a 20-year old female, and the main clinical manifestations were fever, bloody sputum, facial erythema, accompanied by vaginal bleeding. The laboratory test showed that the titer of anti-nuclear antibody （ANA） was 1： 1000, anti-double stranded DNA （ds-DNA） antibody and anti Smith antibody were both positive, and hemoglobin was progressively reduced. The chest CT showed two lung fields displayed more spiral shape and spot shape, nodule density increased shadow, and edge was blur. A diagnosis of SLE combined with DAH was made. She was given plasmapheresis eomhined with glucocorticoid, while giving the human normal immunoglobulin. Thereafter, hemoptysis stopped, facial erythema disappeared, anemia was significantly improved, and now she was given glucocorticoids and got reduction regularly. In our country, 101 cases were reported, and the specific clinical manifestations were described in 83 cases. These patient＇s ANA was all positive, 58 cases were positive for anti ds-DNA antibody, 67 cases presented with hemoptysis, 61 cases presented with fever, 71 cases presented with renal involvement, hemoglobin decreased in 74 cases, there were 80 cases of dyspnea, and 70 cases of hypoxemia. The imaging examination showed pulmonary infiltration. Through the treatment with plasmapheresis, hormone shock, immune inhihitors and other drugs, 10 patients underwent plasma exchange, 7 cases were effective; 29 cases were treated with gamma globulin, 21 cases were effective; 74 patients received high-dose corticosteroid, 47 cases were effective; 21 cases were given mechanical ventilation, 8 cases were effective; 49 cases were treated with immuno-suppressive drugs, 36 cases were effective; 12 cases received the hronchoalveolar lavage, 10 cases were effective. Conclusions DAH is a rare and life-threatening complications of SLE. Early diagnosis, the use of plasmapheresis combined with hormone shock and other supportive treatment, preventing and treating the complications actively, will improve the patient＇s clinical manifestations and prognosis effectively.