[Cardiovascular complications in chronic atrophic polychondritis. Apropos of an anatomo-clinical case. Review of the literature].
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.
Mainguèné
C
C
Service Central d'Anatomie et de Cytologie Pathologiques, Hôtel-Dieu, Paris.
Bouhour
J B
JB
de Lajartre
A Y
AY
Dupon
H
H
D000328:Adult; D002318:Cardiovascular Diseases / Q000209:etiology* / Q000473:pathology; D002908:Chronic Disease; D006801:Humans; D008297:Male; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000473:pathology