Cardiac manifestations of Relapsing Polychondritis
Aortic insufficiency is the most common cardiac manifestation of RPC. After respiratory involvement, it is the most serious complication of RPC. It is usually due to progressive dilatation of the aortic root, which may help in distinguishing it from the aortic insufficiency seen in other conditions.
Less frequent cardiac complications include pericarditis, myocarditis, arrhythmias, coronary aneurysms, valvulitis, and conduction defects. The prevalence of cardiac involvement in RPC in recent cohorts is around 20%, compared with 10% in older studies.
This may potentially reflect enhanced cardiac screening and/or improved sensitivity of current imaging techniques.
Aortic Insufficiency: Patterns of Disease Association
| Pathology | Underlying Condition |
|---|---|
| Valvulitis | Rheumatic fever Rheumatoid arthritis Ankylosing spondylitis∗ Endocarditis Reactive arthritis a Behçet’s syndrome a |
| Congenital | Bicuspid aortic valve |
| Dilatation of valve ring | Marfan’s syndrome Syphilis Relapsing polychondritis Dissecting aneurysm Idiopathic Takayasu’s arteritis Giant cell arteritis Cogan’s syndrome |
a Ankylosing spondylitis, reactive arthritis, and Behcets syndrome can also cause dilatation of the valve ring.
