目的：分析总结弥漫性结缔组织病（CTD）合并弥漫性肺泡出血（DAH）的临床资料，提高对该病的认识。方法回顾分析深圳市人民医院2005年7月至2012年9月确诊的12例弥漫性 CTD 并发 DAH 患者的临床表现、实验室检查、诊治经过和转归。结果DAH 主要表现是咯血、胸闷、呼吸困难、血红蛋白下降；X 线胸片、高分辨率 CT 可出现新发的肺部浸润影。弥漫性 CTD 合并 DAH 的患者中，系统性红斑狼疮（SLE）8例，显微镜下多血管炎（MPA）2例，肉芽肿性多血管炎（GPA）2例；诊断 DAH 时的年龄：SLE（26±8）岁，MPA 分别为12岁和42岁，GPA 为51岁和57岁；诊断 DAH 时的病程：SLE（18±14）d，其中有3例 SLE 患者以 DAH 为首发症状，另外有1例 MPA 患者也以 DAH 为首发症状；SLE 合并 DAH 时 SLE 疾病活动指数（SLEDAI）评分为（18±5）分，抗核抗体呈高滴度，MPA／GPA 患者核周型抗中性粒细胞胞质抗体（p-ANCA）／胞质型抗中性粒细胞胞质抗体（c-ANCA）分别呈阳性；9例患者发生明确感染；共有9例患者接受了激素冲击治疗，10例应用免疫抑制剂，7例行机械通气，2例行血液透析，所有患者病程中均使用抗生素。有1例患者死亡。结论DAH 与弥漫性 CTD 原发病的活动性密切相关，容易导致呼吸衰竭和肺部感染。弥漫性 CTD 合并 DAH 病死率高，应早期诊断，早期治疗。%Objective To provide clues to diagnosis and treatment for diffuse alveolar hemorrhage(DAH)in patients with diffuse connective tissue diseases(CTD).Methods To analyze retrospectively the data of clinical feature,laboratory examination,diagnosis process and curative effect in 12 patients hospitalized in Shenzhen Peo-ple′s Hospital from July 2005 to September 2012.Results The main clinical manifestations of DAH were hemop-tysis,dyspnea and rapid decrease of hemoglobulin and hematocrit (HCT)in peripheral blood.Most patients pres-ented with new alveolar infiltration on chest X-ray and high resolution CT.The data from 8 of the 12 patients with systemic lupus erythematosus(SLE),2 of the 12 patients with microscopic polyangiitis (MPA)and 2 of the 12 pa-tients with granulomatosis with Polyangiitis (GPA)were consistent with the diagnosis of DAH.DAH in SLE oc-curred in younger patients [mean age at the time of diagnosis (26 ± 8)years]and early in the course of disease [median duration of SLE from onset was (18±14)days].While the time of diagnosis in MPA was respectively 12 and 42 year,the figures in GPA was respectively 51 and 57 year,3 SLE patients and 1 MPA patient presented DAH as initial symptoms.At the time of DAH in SLE,the median systemic lupus erythematosus disease activity index (SLEDAI)score was 18±5 and ANA antibody titer elevated markedly.The titer of anti-neutrophil cyto-plasmic antibody (ANCA,p-ANCA/c-ANCA)in patients with MPA and GPA was highly positive.Nine cases of patients occurred clear infection.A total of 10 patients were treated with hormone shock threapy,10 cases with immunosuppressant,7 cases with mechanical ventilation,2 cases with hemodialysis,all patients in the course of the disease were with antibiotics.One patient died.Conclusion DAH in CTD is an acute,serious and frequent life-threatening situation resulting in respiratory failure and pulmonary infection.It is important for CTD patients with DAH to be diagnosed early and treated vigorously.
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