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[系统性红斑狼疮患者的肺出血。临床表现及预后]

[Pulmonary hemorrhage in patients with systemic lupus erythematosus. Clinical manifestations and prognosis].

作者信息

Finucci Curi Pablo, Pierrestegui Matilde, Ortiz Alberto, Ceccato Federico, Paira Sergio

机构信息

Sección de Reumatología, Hospital J. M. Cullen, Santa Fe, Argentina.

Sección de Reumatología, Hospital J. M. Cullen, Santa Fe, Argentina.

出版信息

Med Clin (Barc). 2015 Nov 6;145(9):375-9. doi: 10.1016/j.medcli.2014.07.034. Epub 2014 Nov 13.

Abstract

BACKGROUND AND OBJECTIVE

Pulmonary hemorrhage (PH) in systemic lupus erythematosus (SLE) is a rare but potentially fatal complication that occurs most frequently in the context of active lupus with involvement of other organs. The objective of this study is to report the clinical features and prognosis of patients with SLE who had PH.

METHODS

Patients with SLE (1982 American College of Rheumatology criteria) and PH under monitoring between June 1999 and November 2011 were studied. Demographic, clinical, laboratory, treatment and prognosis data related to PH were analyzed.

RESULTS

Eleven patients with SLE developed 14 episodes of PH. PH was the first manifestation of SLE in 2 patients. The most frequent symptoms and clinical signs were dyspnea, fever and cough, which occurred in 12 (85%), 11 (77%) and 7 (50%) patients, respectively. Hemoptysis was also observed in 5 (35%) episodes, tachycardia in 2 (14%), pallor in one (7%) and chest pain in one (7%). All patients had other concomitant organ involvement, and were treated with glucocorticoids. In addition, intravenous cyclophosphamide was indicated in 12 episodes and plasma exchange in 4. Overall mortality was 64%. Factors associated with mortality were infection, mechanical ventilation and dialysis.

CONCLUSIONS

PH continues to be a rare and severe complication of SLE. Its suspected presence forces us to quickly study these patients, since early diagnosis and aggressive treatment have been shown to improve survival in them.

摘要

背景与目的

系统性红斑狼疮(SLE)合并肺出血(PH)是一种罕见但可能致命的并发症,最常发生于伴有其他器官受累的活动性狼疮患者中。本研究的目的是报告发生PH的SLE患者的临床特征及预后情况。

方法

对1999年6月至2011年11月期间接受监测的SLE(1982年美国风湿病学会标准)合并PH患者进行研究。分析与PH相关的人口统计学、临床、实验室、治疗及预后数据。

结果

11例SLE患者发生了14次PH发作。2例患者中PH是SLE的首发表现。最常见的症状和体征分别为呼吸困难、发热和咳嗽,分别有12例(85%)、11例(77%)和7例(50%)患者出现。5次发作(35%)出现咯血,2例(14%)出现心动过速,1例(7%)出现面色苍白,1例(7%)出现胸痛。所有患者均有其他伴随器官受累,并接受了糖皮质激素治疗。此外,12次发作使用了静脉环磷酰胺,4次进行了血浆置换。总死亡率为64%。与死亡率相关的因素为感染、机械通气和透析。

结论

PH仍然是SLE一种罕见且严重的并发症。怀疑存在PH时,我们必须迅速对这些患者进行研究,因为早期诊断和积极治疗已被证明可提高其生存率。

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