To analyze the clinical features and proteomic profiles of patients with relapsing polychondritis (RP) and respiratory involvement.
Approach:
Patient Enrollment: Seventy-seven RP patients with respiratory involvement were enrolled, and their clinical characteristics were systematically analyzed.
Latent Class Analysis: Latent class analysis (LCA) was used to classify patients based on bronchoscopic findings.
Proteomic Analysis: Proteomic techniques were applied to detect proteins in the serum of healthy controls and RP patients.
Key Findings:
Patients with initial respiratory involvement were less likely to have ear chondritis (14.29% vs 42.86%, p = 0.005), nasal chondritis (24.48% vs 46.43%, p = 0.048), and ocular involvement (16.33% vs 39.29%, p = 0.025).
Three subgroups of patients were identified based on bronchoscopic manifestations, with varying degrees of airway damage.
Patients in groups one and two are defined by cartilage damage of the central airway, with or without peripheral airway involvement, and require more non-invasive ventilation.
Patients in group three are defined by cartilage damage of the entire airway, with a high frequency of ICU admission (p = 0.003), tracheostomy (p <0.001), and rescue (p = 0.001).
Differentially expressed proteins related to complement and neutrophil extracellular trap formation may be involved in the pathogenesis of respiratory-involved RP.
Interpretation:
Clinical manifestations of respiratory-involved RP are nonspecific, and recognition of imaging and bronchoscopic features can facilitate the diagnosis.
Limitations:
The study was retrospective and may have selection bias.
The sample size for proteomic analysis was limited to 35 patients.
Conclusion:
Stratification based on airway involvement patterns may provide insights into the pathogenesis of respiratory-involved RP.