Department of Internal Medicine, Division of Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey.
Department of Internal Medicine, Ankara University Faculty of Medicine, Ankara, Turkey.
Clin Rheumatol. 2021 Oct;40(10):4127-4134. doi: 10.1007/s10067-021-05748-x. Epub 2021 Apr 27.
To report the clinical characteristics of pulmonary artery involvement (PAI) in patients with Behçet's syndrome (BS) and to define the predictors of relapses.
We performed retrospective analysis of BS patients with PAI who fulfilled international study group criteria. Among 460 patients with vascular Behçet's syndrome (VBS), 66 were diagnosed with PAI. For final analyses, 61 patients with PAI were included who had at least 2 follow-up visits (72.1% male, mean age at BS diagnosis 29.34 ± 10.1 years). The patient data were recorded. Relapse was defined as the reoccurrence of vascular event in any vascular structure. Factors associated with relapse were assessed by logistic regression analysis.
There were no differences considering demographic and clinical features of the patients with and without PAI in the VBS group, except that intracardiac thrombosis was more common in the patients with PAI (19.7% vs 0.3%). Among 61 patients, 50 (82.0%) had isolated pulmonary artery thrombosis (PAT), whereas 11 (18.0%) had pulmonary artery aneurysm with or without PAT. Twenty-four (39.3%) patients experienced vascular relapse during median follow-up of 65.9 (Q1-Q3: 20.1-109.0) months. To define the factors associated with relapses, patients with isolated PAT were analysed. On multivariable logistic regression analysis, older age at BS diagnosis and anticoagulation usage seemed to be protective (OR: 0.92, 95% CI 0.86-1.02, OR: 0.34, 95% CI 0.09-1.33, respectively).
Our results indicate a higher frequency of intracardiac thrombosis in BS patients with PAI and possible efficacy of anticoagulation usage in preventing relapses. Key Points • This study shows that intracardiac and intracranial thromboses are seen more frequently in patients with PAI and the prevalence of pulmonary artery thrombosis has been increasing in the case of PAI. Furthermore, our report indicates that anticoagulation might be effective in preventing further vascular relapses.
报告白塞病（BS）患者肺动脉受累（PAI）的临床特征，并确定复发的预测因素。
我们对符合国际研究组标准的白塞病合并 PAI 患者进行了回顾性分析。在 460 例血管性白塞病（VBS）患者中，66 例诊断为 PAI。最终分析中，纳入了 61 例至少有 2 次随访的 PAI 患者（72.1%为男性，BS 诊断时的平均年龄为 29.34±10.1 岁）。记录患者数据。复发定义为任何血管结构中血管事件的再次发生。通过 logistic 回归分析评估与复发相关的因素。
在 VBS 组中，PAI 患者与无 PAI 患者在人口统计学和临床特征方面没有差异，除了 PAI 患者更常见心内血栓（19.7%比 0.3%）。在 61 例患者中，50 例（82.0%）单纯肺动脉血栓形成（PAT），11 例（18.0%）有肺动脉瘤伴或不伴 PAT。在中位随访 65.9（Q1-Q3：20.1-109.0）个月期间，24 例（39.3%）患者发生血管再发。为了确定与复发相关的因素，分析了单纯 PAT 患者。多变量 logistic 回归分析显示，BS 诊断时年龄较大和抗凝治疗似乎具有保护作用（OR：0.92，95%CI 0.86-1.02；OR：0.34，95%CI 0.09-1.33）。
我们的结果表明，PAI 患者 BS 中更常见心内和颅内血栓形成，抗凝治疗可能有效预防复发。关键点：• 本研究表明，PAI 患者更常出现心内和颅内血栓形成，PAI 患者的肺动脉血栓形成患病率呈上升趋势。此外，我们的报告表明抗凝治疗可能有效预防进一步的血管再发。
