Unsuspected Cardiovascular Involvement in Relapsing Polychondritis
A Case of Aortitis With Critical Coronary Artery Stenosis Secondary to Relapsing Polychondritis
A 51-year-old man presented with a prodrome of arthralgia, intermittent oral ulceration, and raised inflammatory markers. He was extensively investigated; however, no firm diagnosis could be established, and he remained under regular surveillance by rheumatology and infectious disease services. After 12 months, he presented with painful ear swelling. Clinical examination revealed a swollen tender ear (Figure 1) and a soft diastolic heart murmur. Serum inflammatory markers were raised, with a c-reactive protein (CRP) of 28 mg/L and an erythrocyte sedimentation rate (ESR) of 25 mm/h.
Biopsy of affected cartilage showed characteristic features of auricular chondritis (Figure 2). Echocardiogram (Movie I in the Data Supplement) revealed mild aortic incompetence, a left ventricular ejection fraction of 60%, aortic root dilatation, and an incidental patent foramen ovale (Movie II in the Data Supplement). Computed tomography (CT) of the thorax demonstrated no tracheal abnormality; however, aortic root dilatation of 4.6 cm was noted (Figure 3). A fasting positron emission tomography-CT (PET-CT) showed increased F-18 fluorodeoxyglucose accumulation in the ascending aorta (Figure 4), and a diagnosis of relapsing polychondritis with secondary aortitis with structural aortic dilatation was made.