Relapsing polychondritis presenting with complete heart block

Relapsing polychondritis (RP) is a rare autoimmune disease characterized by recurrent inflammation of cartilagenous structures, including the ears, nose, trachea, and joints. Cardiovascular involvement is relatively common, but involvement of the conduction system leading to various degrees of heart block is a rare and late manifestation of the disease usually requiring a pacemaker. We report here a patient with RP who presented with complete heart block that resolved promptly after treatment with prednisone. To our knowledge, this is the first reported case of RP with abnormal cardiac conduction on initial presentation and a rapid response to anti-inflammatory treatment. Heart block during the phase of active inflammation may respond to corticosteroid treatment as in our patient without the need for cardiac pacing.