青年狼疮肾炎合并弥漫性肺泡出血的临床特点

Clinical characteristics of diffuse alveolar hemorrhage in young patients with lupus nephritis

目的明确青年狼疮肾炎合并弥漫性肺泡出血的临床特点。方法分析我院收治的4例青年狼疮肾炎合并弥漫性肺泡出血患者临床表现、体格检查、实验室检查、影像学检查及特殊辅助检查结果和治疗转归等情况。结果4例均为女性。年龄18-25岁，平均20．5岁，平均病程26个月，3例为病情复发后出现弥漫性肺泡出血。其中发热、咯血、呼吸困难是最多见的症状（50％～100％）。系统性红斑狼疮疾病活动指数（SLEDAI）评分显示病情活动期〉9分的2例。肾脏病理提示3例为Ⅳ型，1例为Ⅱ型。影像学结果发现所有患者双肺均有弥漫性肺泡浸润影。支气管肺泡灌洗液呈血性。3例患者在接受激素及免疫抑制剂治疗后症状好转或消失。结论弥漫性肺泡出血是青年狼疮肾炎的严重并发症，缺乏早期的特异性诊断指标，强化免疫抑制治疗有望改善患者的预后。

Objective To determine the clinical characteristics of diffuse alveolar hemorrhage （DAH） in youthful patients with lupus nephritis. Methods Four DAH inpatients with lupus nephritis were included for reviewing clinical manifestation, physical examination, lab test, imaging analysis and treatment response. Results All patients were female with average age 20.5 years old. Duration of the illness was 26 months. In 3 patients DAH occurred during lupus nephritis relapse. Dyspnea, hemoptysis and fever were the most common symptoms （50% - 100% ）. Two patients were with active disease （SLEDAI score〉9）. Renal pathology revealed 3 patients with class Ⅳ and 1 with class Ⅱ. Imaging results showed diffuse pulmanery infiltration lesions. Bronchial lavage fluid showed hemorrhage. Three patients recovered after treatment with corticosteroid and intravenous cyclophosphamide. Conclusion Diffuse alveolar hemorrhage is a rare yet se_rious complication of lupus nephritis. Aggressive immunosuppressive therapy should be started early for a favorable outcome.