目的：提高对以弥漫性肺泡出血（DAH）为首发症状的系统性红斑狼疮（SLE）的认识。方法报道1例SLE并DAH且以DAH为首发症状的患者临床资料，并复习相关文献。结果通过病例分析，总结其临床特点、发病机制、诊断标准、治疗方法。结论以DAH为首发症状的SLE发病率低，但病情严重，早期诊断及强化治疗有望改善预后。“,”Objective To improve the understanding of systemic lupus erythematosus (SLE) complicated with diffuse alveolar hemorrhage (DAH). Methods One case of SLE complicated with DAH, further more DAH as the ifrst symptom, was reported and the related literatures were reviewed. Results Through analyzing the case, the clinical characteristics, psychotic mechanism, diagnosis and treatment of SLE complicated with DAH were summarized. Conclusion The morbidity of SLE start with DAH was very low, but the pathogenetic condition was severe. Maybe the early diagnosis and intensive treatment can achieve the favorable outcome.