系统性红斑狼疮患者的弥漫性肺泡出血:西班牙风湿病学会狼疮登记册(RELESSER)的数据。

IF 3.2 3区 医学 Q2 RHEUMATOLOGY
Rheumatology International Pub Date : 2024-11-01 Epub Date: 2024-08-24 DOI:10.1007/s00296-024-05684-4
María Jesús Garcia-Villanueva, Sandra Garrote-Corral, Jose María Pego-Reigosa, Norman Jiménez Otero, Esther Uriarte Isazelaia, Alejandro Olivé Marqué, Clara Sangüesa Gómez, Mercedes Freire González, Elena Aurrecoechea Aguinaga, Enrique Raya Álvarez, Eva Tomero Muriel, Carlos Montilla Morales, María Galindo Izquierdo, Jaime Calvo-Alén, Raúl Menor-Almagro, Belén Serrano Benavente, Julia Martinez-Barrio, Jose Angel Hernández-Beriain, Mónica Ibañez Barceló, Gema Bonilla Hernan, Jose Rosas, Eva Salgado Pérez, Antonio Fernández-Nebro, Iñigo Rua-Figueroa
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引用次数: 0

摘要

简介:弥漫性肺泡出血(DAH 弥漫性肺泡出血(DAH)是系统性红斑狼疮(SLE)患者的一种罕见并发症,死亡率很高。早期诊断和治疗对改善患者预后至关重要。目的是确定西班牙系统性红斑狼疮患者队列中DAH患者的特征及其死亡率: 方法:纳入RELESSER(西班牙风湿病学会狼疮登记册)中至少确诊过一次DAH的患者。分析了流行病学、临床和实验室特征: 结果:4024 名患者被纳入 RELESSER 登记册,其中 37 人(0.9%)至少有一次 DAH 病发记录。只有 14 名患者的进一步数据可以进行分析。其中,92.9%的患者为女性,4名患者(28.6%)的DAH与系统性红斑狼疮同时出现。80%以上的患者有肾脏受累和血小板减少。最常见的表现是呼吸困难(85.7%)和低氧血症(100%),6 名患者(46.2%)出现典型的三联征:咯血、贫血和肺部浸润。最常用的治疗方法是糖皮质激素(85.7%)和环磷酰胺(69.2%);5 名患者(35.7%)使用了血浆置换术,8 名患者(57.1%)接受了静脉注射免疫球蛋白;12 名患者(85.7%)需要入住重症监护室,5 名患者(35.7%)死亡。吸烟、狼疮性肾炎(LN)病史、并发感染和环磷酰胺治疗在死亡患者中更为常见: DAH在系统性红斑狼疮患者中较为罕见;多达三分之一的患者可能在发病初期就出现DAH。某些因素,如吸烟、LN病史、环磷酰胺治疗或并发感染,在预后不良的患者中更为常见。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Diffuse alveolar hemorrhage in patients with systemic lupus erythematosus: data from the Spanish society of rheumathology Lupus Register (RELESSER).

Introduction:  Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE.

Methods:  Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed.

Results:  4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died.

Conclusions:  DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome.

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来源期刊
Rheumatology International
Rheumatology International 医学-风湿病学
CiteScore
7.30
自引率
5.00%
发文量
191
审稿时长
16. months
期刊介绍: RHEUMATOLOGY INTERNATIONAL is an independent journal reflecting world-wide progress in the research, diagnosis and treatment of the various rheumatic diseases. It is designed to serve researchers and clinicians in the field of rheumatology. RHEUMATOLOGY INTERNATIONAL will cover all modern trends in clinical research as well as in the management of rheumatic diseases. Special emphasis will be given to public health issues related to rheumatic diseases, applying rheumatology research to clinical practice, epidemiology of rheumatic diseases, diagnostic tests for rheumatic diseases, patient reported outcomes (PROs) in rheumatology and evidence on education of rheumatology. Contributions to these topics will appear in the form of original publications, short communications, editorials, and reviews. "Letters to the editor" will be welcome as an enhancement to discussion. Basic science research, including in vitro or animal studies, is discouraged to submit, as we will only review studies on humans with an epidemological or clinical perspective. Case reports without a proper review of the literatura (Case-based Reviews) will not be published. Every effort will be made to ensure speed of publication while maintaining a high standard of contents and production. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.
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