Clinical Characteristics of Our Follow-Up Patients with the Rare Disease Relapsing Polychondritis: A Single-Center Study

Abstract

Relapsing polychondritis (RP) is an immune-mediated, systemic inflammatory and degenerative disease that affects cartilaginous structures particularly the ears, nose, eyes, joints, and respiratory tract and other tissues in the body. RP targets non-cartilaginous structures such as skin, kidney, heart, and the central nervous system in addition to cartilage-containing structures. Since it is a rare disease, data on its epidemiology are insufficient. It is prevalent between the ages of 40 and 60. Men and women are affected equally. Clinical characteristics vary among patients. The disease's rarity and broad clinical spectrum frequently result in misdiagnosis or delayed diagnosis. To prevent related complications and death, and to improve prognosis, early diagnosis and timely treatment of RP are crucial. Glucocorticoids, dapsone, disease-modifying antirheumatic drugs (DMARDs), and biologics are available as treatment options. The prognosis, such as the clinical presentation, varies depending on the extent of organ damage. 10 patients with RP had a mean onset age of 49.5±4.1. The median time between symptom onset and diagnosis was 3 months (2-60). 80% of the patients were male. The most frequent clinical manifestation is auricular chondritis (100%). During treatment, oral prednisolone was administered to all patients at least once. Intravenous methylprednisolone was administered to two patients. Due to ineffectiveness of DMARDs, one patient was switched to infliximab. A patient died due to pneumosepsis. This article aims to increase clinicians' awareness of this rare disease that can affect multiple systems by providing an overview of its pathogenesis, clinical course, diagnosis, and treatment.