Unsuspected Cardiovascular Involvement in Relapsing Polychondritis

Abstract

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. Figure 1. Tender, erythematous left ear. 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. Figure 2. Left ear biopsy identified cartilage with perichondrial inflammatory granulation tissue. Figure 3. Coronal computed tomography of thorax with contrast demonstrating aortic root dilatation of 4.6 cm at the level of the sinuses with mural …