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系统性红斑狼疮合并弥漫性肺泡出血患者的特征:单中心经验的临床特征与结局

Characteristics of Systemic Lupus Erythematosus Patients with Diffuse Alveolar Hemorrhage: Clinical Features and Outcomes from a Single-Center Experience.

作者信息

Dziedzic Radosław, Korkosz Mariusz, Kosałka-Węgiel Joanna

机构信息

Jagiellonian University Medical College, Doctoral School of Medical and Health Sciences, św. Łazarza 16, 31-530 Kraków, Poland.

Jagiellonian University Medical College, Department of Rheumatology and Immunology, Jakubowskiego 2, 30-688 Kraków, Poland.

出版信息

J Clin Med. 2025 Aug 8;14(16):5614. doi: 10.3390/jcm14165614.

Abstract

: Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening complication that might occur in the course of systemic lupus erythematosus (SLE), presenting with acute respiratory symptoms, a rapid drop in hemoglobin, and diffuse pulmonary infiltrates. Despite various studies, clinical and laboratory risk factors for DAH in SLE remain unclear due to small cohort sizes and inconsistent findings. : We analyzed the medical records of all adult SLE patients treated at the University Hospital in Kraków, Poland, from 2012 to 2022, to look for patients with DAH. : In a cohort of 1039 SLE patients, DAH was confirmed in five cases (0.48%), all presenting with respiratory symptoms and significant hemoglobin drops. No patients required intensive care unit admission or mechanical ventilation, and all survived the 5-year follow-up after receiving immunosuppressive therapy including glucocorticosteroids and cyclophosphamide, and also rituximab in one case. Common features included constitutional symptoms, hematologic and renal involvement, and frequent presence of antiphospholipid antibodies, with antiphospholipid syndrome diagnosed in three patients (60%). All patients had positive antinuclear antibodies, with the presence of anti-dsDNA and anti-SSA antibodies, each present in 3 out of 5 cases. : In conclusion, early recognition and aggressive treatment of DAH in SLE patients, who often present other medical comorbidities as hematological, renal, and cardiovascular manifestations, is critical for improving long-term outcomes.

摘要

弥漫性肺泡出血(DAH)是一种罕见但危及生命的并发症,可能发生在系统性红斑狼疮(SLE)病程中,表现为急性呼吸道症状、血红蛋白迅速下降和弥漫性肺部浸润。尽管进行了各种研究,但由于队列规模小且研究结果不一致,SLE中DAH的临床和实验室危险因素仍不明确。我们分析了2012年至2022年在波兰克拉科夫大学医院接受治疗的所有成年SLE患者的病历,以寻找DAH患者。在1039例SLE患者队列中,确诊5例DAH(0.48%),均表现出呼吸道症状和显著的血红蛋白下降。没有患者需要入住重症监护病房或进行机械通气,所有患者在接受包括糖皮质激素和环磷酰胺在内的免疫抑制治疗后均存活了5年随访,其中1例还接受了利妥昔单抗治疗。常见特征包括全身症状、血液学和肾脏受累,以及抗磷脂抗体频繁出现,3例患者(60%)诊断为抗磷脂综合征。所有患者抗核抗体均为阳性,5例中有3例同时存在抗双链DNA和抗SSA抗体。总之,对于常伴有血液学、肾脏和心血管表现等其他合并症的SLE患者,早期识别和积极治疗DAH对于改善长期预后至关重要。

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