Later Selma, Abdelbagi Muzan, Zaaroura Amjad, Sherif Reda Mohamed, Aldebese Khaled Mohammed Yehia Alsayed Abdel Salam, Eisa Mostafa Mohamed, Badawy Mohamed H
Department of Anesthesia and Critical Care Medicine, Al Qassimi Hospital, Emirates Health Services, Sharjah, United Arab Emirates.
Department of Critical Care Medicine, Al Kuwait Hospital, Emirates Health Services, Sharjah, United Arab Emirates.
Am J Case Rep. 2025 Jun 21;26:e947096. doi: 10.12659/AJCR.947096.
BACKGROUND Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE), with a mortality rate of up to 80%. It is seldom the initial manifestation of SLE, making early diagnosis and treatment challenging. DAH typically presents with hemoptysis, abrupt anemia, and diffuse pulmonary infiltrates, although up to 50% of cases lack hemoptysis, complicating diagnosis. CASE REPORT We present a case of a 23-year-old woman with no prior SLE diagnosis who presented with dyspnea, cough, and fever. Initial investigations revealed normocytic normochromic anemia (hemoglobin 7.3 g/dL), acute kidney injury (creatinine 323 µmol/L), and bilateral pulmonary infiltrates on imaging. DAH was confirmed via bronchoscopy, which showed hemorrhagic bronchoalveolar lavage fluid, and high-resolution computed tomography of the chest revealed pulmonary consolidations with peripheral sparing. Autoimmune workup confirmed SLE, with positive antinuclear antibodies, anti-dsDNA antibodies, and low complement levels. The patient was treated with high-dose corticosteroids, rituximab, and plasma exchange. Despite initial stabilization and weaning from mechanical ventilation, she developed refractory pancytopenia, posterior reversible encephalopathy syndrome, and multi-organ failure, leading to her death on hospital day 48. CONCLUSIONS This case stresses the value of a high index of suspicion for DAH in young women with respiratory symptoms, even in the absence of a known SLE diagnosis. Early diagnostic measures, including bronchoscopy and imaging, are critical for timely intervention. Aggressive immunosuppressive therapy and plasma exchange can stabilize patients temporarily, but refractory cases remain challenging. A multidisciplinary approach is essential to improve outcomes in this high-mortality condition.
背景 弥漫性肺泡出血(DAH)是系统性红斑狼疮(SLE)一种罕见但危及生命的并发症,死亡率高达80%。它很少是SLE的初始表现,这使得早期诊断和治疗具有挑战性。DAH通常表现为咯血、急性贫血和弥漫性肺部浸润,尽管高达50%的病例无咯血症状,这使诊断变得复杂。病例报告 我们报告一例23岁女性,既往无SLE诊断,出现呼吸困难、咳嗽和发热。初始检查发现正细胞正色素性贫血(血红蛋白7.3 g/dL)、急性肾损伤(肌酐323 µmol/L),影像学检查显示双侧肺部浸润。通过支气管镜检查确诊为DAH,支气管肺泡灌洗显示出血性灌洗液,胸部高分辨率计算机断层扫描显示肺部实变伴外周肺野 spared 。自身免疫检查确诊为SLE,抗核抗体、抗双链DNA抗体阳性,补体水平降低。患者接受了大剂量糖皮质激素、利妥昔单抗和血浆置换治疗。尽管最初病情稳定并脱机,但她出现了难治性全血细胞减少、后部可逆性脑病综合征和多器官功能衰竭,于住院第48天死亡。结论 本病例强调对于有呼吸道症状的年轻女性,即使无已知SLE诊断,也应高度怀疑DAH。包括支气管镜检查和影像学检查在内的早期诊断措施对于及时干预至关重要。积极的免疫抑制治疗和血浆置换可使患者暂时稳定,但难治性病例仍然具有挑战性。多学科方法对于改善这种高死亡率疾病的治疗结果至关重要。 (注:原文中“peripheral sparing”表述可能有误,推测可能是“peripheral sparing”,意为外周肺野 spared ,这里暂按此翻译)
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