Department of Internal Medicine, Rouen University Hospital, 1, rue de Germont, 76000 Rouen, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen University Hospital, 76000 Rouen, France.
Department of Rheumatology, and French Reference Center for Rare Autoimmune and Systemic Diseases, hôpital de Hautepierre, 1, avenue Molière, BP 83049, 67098 Strasbourg cedex, France; INSERM 1109, Immunorhumatologie moléculaire, hôpital de Hautepierre, 67098 Strasbourg cedex, France.
Joint Bone Spine. 2018 May;85(3):345-351. doi: 10.1016/j.jbspin.2017.05.009. Epub 2017 May 17.
To assess prevalence of aortic involvement in relapsing polychondritis (RP) patients; to evaluate clinical features and long-term outcome of RP patients exhibiting aortitis, aortic ectasia and/or aneurysm.
One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers.
Eleven patients (6.4%) had aortic involvement, occurring within a median time of 2 years after RP diagnosis. CT-scan showed isolated aortitis (n=2); the 9 other patients exhibited: aortitis and aortic aneurysm (n=2) or ectasia (n=1), isolated aortic aneurysm (n=4) or ectasia (n=2); aortic localizations were as follows: thoracic (n=6), abdominal (n=2), thoracic and abdominal (n=4) aorta. Patients exhibited: resolution (n=3) improvement (n=3), stabilization (n=4) or deterioration (n=1) of aortic localization. Five patients experienced recurrence of aortic localization; one patient died of aortic abdominal aneurysm rupture. Predictive factors of death related to aortic complications were: aortitis on CT-scan, higher median levels of erythrocyte sedimentation rate. Predictive parameters of aortic relapses were: aortitis on CT-scan and involvement of the abdominal aorta.
This study underlines that aortic involvement is severe in RP. Furthermore, we suggest that RP patients exhibiting poor prognostic factors, including panaortitis and higher values of ESR, may require more aggressive therapy.
评估复发性多软骨炎（RP）患者主动脉受累的发生率；评估表现为主动脉炎、主动脉扩张和/或动脉瘤的 RP 患者的临床特征和长期预后。
172 例 RP 患者接受了主动脉计算机断层扫描（CT）检查；他们在 3 家医疗中心就诊。
11 例（6.4%）患者存在主动脉受累，在 RP 诊断后中位时间 2 年内发生。CT 扫描显示孤立性主动脉炎（n=2）；其余 9 例患者表现为：主动脉炎和主动脉瘤（n=2）或扩张（n=1）、孤立性主动脉瘤（n=4）或扩张（n=2）；主动脉定位如下：胸主动脉（n=6）、腹主动脉（n=2）、胸主动脉和腹主动脉（n=4）。患者表现为：主动脉定位的缓解（n=3）、改善（n=3）、稳定（n=4）或恶化（n=1）。5 例患者出现主动脉定位复发；1 例患者死于腹主动脉瘤破裂。与主动脉并发症相关的死亡的预测因素为：CT 扫描上的主动脉炎、红细胞沉降率中位数较高。主动脉复发的预测参数为：CT 扫描上的主动脉炎和腹主动脉受累。
本研究强调了 RP 中主动脉受累的严重性。此外，我们建议表现出预后不良因素（包括全主动脉炎和更高的 ESR 值）的 RP 患者可能需要更积极的治疗。
