Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: analysis of 126 Chinese patients

Objective To explore the clinical patterns of relapsing polychondritis(RPC) to improve clinicians’ comprehensive understanding of the disease. Methods The electronic records of RPC patients that were hospitalized and followed-up by rheumatologists at our hospital between January 1, 2008 and August 31,2019 were retrospectively analyzed. Results Sixty-six males and 60 femaleswere included with onset age of 47.1±13.8 years and followed up for a median of 18 months. Eighty-seven patients (69.1%) did not met with the traditional criteria.Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r=-0.75，P<0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%) and patients with Ear pattern and Airway pattern were subdivided as limited and systemic form of RPC (27.8%with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern andsome Airway-Ear negative patternpatients progressed into Ear pattern. Most of the limited RPC patientsremained limited form during follow-up whilesome patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative lower inflammatory status. Conclusion RPC patients can be categorized as 4 differentclinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement.Limited form of RPC is not uncommon in Chinese patients and traditional criteria of RPC are not suitable for early diagnosis.