青少年狼疮性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

目的探讨青少年狼疮性肾炎合并弥漫性肺泡出血(DAH)的临床特点。方法总结4例青少年狼疮性肾炎合并DAH患者的临床资料、特殊检查和治疗转归等情况。结果4例均为女性。病程平均26个月,3例为复发病例。发热、咯血、呼吸困难最多见(50%~100%)。2例系统性红斑狼疮疾病活动指数(SLEDAI)>9分。肾脏病理3例为Ⅳ型,1例为Ⅱ型。影像学结果示双肺均有弥漫性肺泡浸润影。支气管肺泡灌洗液呈血性。3例接受激素及免疫抑制剂治疗后好转。结论青少年狼疮性肾炎合并DAH病情严重,难以早期诊断,强化治疗有望改善预后。

Objective To determine the clinical characteristics of diffuse alveolar hemorrhage （DAH） in young patients with lupus nephritis. Methods Four patients of DAH with lupus nephritis were included for reviewing clinical manifestation, physical examination, lab t^t, image analysis and treatment response. Results All patients were female with average age 20.5 yearn old. Duration of the illness was 26 months. Three patients of DAH occurred during lupus nephritis relapse. Dyspnea, hemop- tysis and fever were the most common symptoms （50% - 100% ）. Two patients were with active dis- ease （SLEDAI score 〉 9）.Renal pathology revealed 3 patients with class Ⅳ, 1 patient with class Ⅱ. Image results showed diffuse pulmonary infiltration lesions. Bronchial lavage fluid was hemorrhage. Three patients recovered after treatment with corticosteroid and intravenous cyclophosphamide therapy. Conclusions Diffuse alveolar hemorrhage is rare serious complications of lupus nephritis. Aggressive immunosuppressive therapy should be started early for a favorable outcome.