Maëlle Le Besnerais1, Laurent Arnaud2, Jonathan Boutémy3, Boris Bienvenu3, Hervé Lévesque1, Zahir Amoura4, Isabelle Marie5. 1. 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. 2. 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. 3. Department of Internal Medicine, Caen University Hospital, 14003 Caen, France. 4. Department of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, 75013 Paris, France; Pierre-and-Marie-Curie University, UPMC University Paris 6, 75013 Paris, France. 5. 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. Electronic address: isabelle.marie@chu-rouen.fr.
Abstract
OBJECTIVE: 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. METHODS: One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers. RESULTS: 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. CONCLUSIONS: 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.
OBJECTIVE: 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. METHODS: One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers. RESULTS: 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. CONCLUSIONS: 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.