Aortic involvement in a patient with relapsing polychondritis. Clinical observation

Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by inflammation and destruction of cartilage in various organs. Aortic involvement in RP is relatively rare, occurring in 6.7–9.7% of cases, and is associated with a high risk of mortality (27.3–41.3%) and surgical interventions (65%). Typically, cardiac and aortic involvement in patients with RP is identified approximately five years after the onset of the first symptoms, with 19% of cases being asymptomatic. There are currently no universally accepted standards for screening, diagnosis, and treatment of aortic involvement in RP. We present a clinical case of a patient with RP, aortic involvement, and the development of an aneurysm, who underwent sequential therapy with cyclophosphamide and rituximab. This treatment approach allowed for the control of disease activity and a reduction in glucocorticoid dosage.