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[系统性红斑狼疮中的弥漫性肺泡出血]

[Diffuse alveolar hemorrhage in systemic lupus erythematosus].

作者信息

Kovács L, Simon J, Kovács A, Pócsik A, Boros I, Iványi B, Pokorny G

机构信息

I. Belgyógyászati Klinika, Szent-Györgyi Albert Orvostudományi Egyetem, Szeged.

出版信息

Orv Hetil. 2000 Jan 23;141(4):179-83.

Abstract

Of the 120 systemic lupus erythematosus (SLE) patients treated by the authors, two have developed diffuse alveolar haemorrhage. The authors' objective is to present this rare, but severe manifestation. Patients 1 and 2 were 66- and 22-year old women, respectively. Both had SLE with multi-organ involvements including diffuse proliferative lupus nephritis. Before the diagnosis of the disease, both patients had experienced pneumonitis that resolved on corticosteroid treatment. Soon after the diagnosis, respiratory failure, haemoptoea and acute anaemia developed, accompanied by a rapid deterioration in the general condition. Chest radiographs revealed bilateral, diffuse, alveolar infiltrates. The pulmonary haemorrhage temporarily ceased in response to corticosteroid treatment, but both patients later died in consequence of active SLE and mixed bacterial and fungal sepsis. Post mortem examination demonstrated fibrosing alveolitis and alveolar bleeding in Patient 1, and an immune complex deposition-induced alveolocapillary inflammation with alveolar haemorrhage in Patient 2. Diffuse alveolar haemorrhage is a life-threatening manifestation of SLE. Its onset may be preceded by episodes of pneumonitis resolving on corticosteroid treatment. An active diagnostic workup, intensive observation and aggressive immunosuppressive treatment are the cornerstones of the management. The early detection and the active treatment of secondary infections are obligatory. The authors consider the most difficult challenge to be the optimum coordination of the above treatment modalities.

摘要

在作者治疗的120例系统性红斑狼疮(SLE)患者中,有2例发生了弥漫性肺泡出血。作者的目的是介绍这种罕见但严重的表现。患者1和患者2分别为66岁和22岁女性。两人均患有累及多器官的SLE,包括弥漫性增殖性狼疮性肾炎。在疾病诊断之前,两名患者均经历过肺炎,经皮质类固醇治疗后痊愈。诊断后不久,出现呼吸衰竭、咯血和急性贫血,同时全身状况迅速恶化。胸部X线片显示双侧弥漫性肺泡浸润。经皮质类固醇治疗后,肺出血暂时停止,但两名患者后来均因活动性SLE以及混合性细菌和真菌败血症而死亡。尸检显示,患者1有肺纤维化和肺泡出血,患者2有免疫复合物沉积引起的肺泡毛细血管炎症伴肺泡出血。弥漫性肺泡出血是SLE的一种危及生命的表现。其发病前可能有经皮质类固醇治疗后痊愈的肺炎发作。积极的诊断检查、密切观察和积极的免疫抑制治疗是治疗的基石。必须尽早发现并积极治疗继发感染。作者认为最困难的挑战是上述治疗方式的最佳协调。

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