Mainguèné C, Bouhour J B, de Lajartre A Y, Dupon H
Service Central d'Anatomie et de Cytologie Pathologiques, Hôtel-Dieu, Paris.
Ann Cardiol Angeiol (Paris). 1991 Feb;40(2):97-102.
The case of a man, 25 years of age and presenting with chronic atrophic polychondritis (CAP), complicated by a complete atrioventricular block, double mitral valve incompetence and aneurysm of the ascending aorta, offered the possibility of investigating the various clinical manifestations and cardiovascular complications of this common disorder. CAP is a connectivitis of unknown etiology, it corresponds to ubiquitous and recurrent cartilage inflammation, leading to characteristic chondritis of the ears and nose, joint disease and laryngo-trachco-bronchial disorders. Other systemic impact is seen at sites containing high levels of proteoglycans, such as the eye, inner ear and cardiovascular system. Respiratory problems are the main cause of death, but cardiovascular effects occur in 25% of cases and constitute the second most frequent cause of mortality. These effects consist mainly of aortic and/or mitral valve incompetence. Annular dilatation, which is often associated with ectasia of the ascending aorta, is the main cause of aortic incompetence. Several cases of isolated AVB or AVB secondary to Al have been reported. Aneurysms develop along the aorta and the large and medium caliber arteries (sub-clavicular, coronary, mesenteric arteries). These are characterized by destruction of the elastic fibers and a reduction in the proteoglycan content of the walls, which is also observed when dystrophy of the cartilage occurs. Other vascular disorders reported include arteritis of the legs, superficial migratory varices and vascularitis, which in some cases gave rise to skin, renal or neurological reactions.
一名25岁男性患者，患有慢性萎缩性多软骨炎（CAP），并发完全性房室传导阻滞、二尖瓣关闭不全和升主动脉瘤，这为研究这种常见疾病的各种临床表现和心血管并发症提供了可能。CAP是一种病因不明的结缔组织炎，表现为普遍存在且反复发作的软骨炎症，导致耳部和鼻部特征性的软骨炎、关节疾病以及喉气管支气管疾病。在含有高水平蛋白聚糖的部位，如眼睛、内耳和心血管系统，也可见其他系统性影响。呼吸问题是主要死因，但心血管影响在25%的病例中出现，是第二常见的死亡原因。这些影响主要包括主动脉和/或二尖瓣关闭不全。环状扩张常与升主动脉扩张相关，是主动脉关闭不全的主要原因。已报道了几例孤立性房室传导阻滞或继发于Al的房室传导阻滞病例。动脉瘤沿着主动脉以及大中型动脉（锁骨下动脉、冠状动脉、肠系膜动脉）发展。其特征是弹性纤维破坏和血管壁蛋白聚糖含量减少，软骨营养不良时也会出现这种情况。其他报道的血管疾病包括腿部动脉炎、浅表游走性静脉曲张和血管炎，在某些情况下会引发皮肤、肾脏或神经反应。
