Monsel G, Maubec E, Picard-Dahan C, Brocheriou I, Henry Feugeas M-C, Kieffer E, Francès C, Crickx B
Service de Dermatologie, AP-HP, Groupe Hospitalier Bichat Claude Bernard, Université Paris 7 Denis-Diderot, Paris.
Ann Dermatol Venereol. 2007 Jun-Jul;134(6-7):552-4. doi: 10.1016/s0151-9638(07)89267-2.
Although rare, cardiovascular involvement is the second most frequent cause of mortality in chronic relapsing polychondritis behind tracheobronchial tree chondritis. The most frequent cardiovascular complications are valvulopathy and aortic aneurysm.
We report a case of chronic relapsing polychondritis with multiple aortic aneurysms that were clinically silent but continued to progress despite systemic corticosteroids and immunosuppressive therapy.
Progression of aortic aneurysms and extravascular disease do not appear to be correlated. Although the disease may appear to be in remission, vascular lesions can continue to progress independently. This case shows that medical treatment has little effect on the progression of these aneurysms. Consequently, it is necessary to opt for surgical therapy at the opportune moment.
心血管受累虽然罕见，但却是复发性多软骨炎患者仅次于气管支气管树软骨炎的第二大常见死亡原因。最常见的心血管并发症是瓣膜病和主动脉瘤。
我们报告一例复发性多软骨炎合并多发主动脉瘤的病例，这些动脉瘤临床上无明显症状，但尽管接受了全身糖皮质激素和免疫抑制治疗仍持续进展。
主动脉瘤的进展与血管外疾病似乎并无关联。尽管疾病可能看似缓解，但血管病变仍可独立进展。该病例表明，药物治疗对这些动脉瘤的进展几乎没有作用。因此，有必要在适当的时候选择手术治疗。
