Clinical Manifestations and Treatment in Patients With Relapsing Polychondritis: A Multicenter Observational Cohort...

Area of Science:

• Rheumatology
• Immunology
• Rare Diseases

Background:

• Relapsing polychondritis (RP) is a rare, multisystemic autoimmune disorder characterized by inflammation of cartilaginous tissues.
• The disease heterogeneity and lack of established treatment protocols present significant challenges for patient management.

Purpose of the Study:

• To describe the clinical manifestations and organ damage in a cohort of patients with relapsing polychondritis.
• To analyze different treatment approaches and their association with disease outcomes.

Main Methods:

• An observational, multicenter cohort study involving adult patients diagnosed with RP.
• Data collection included clinical manifestations, organ damage, and medication history.
• Treatments were categorized into three groups based on drug classes: glucocorticoids (GCs) alone or no drugs, nonbiologic immunosuppressives (IS) with or without GCs, and JAK inhibitors (JAKis) or biologic IS drugs with or without other treatments.

Main Results:

• The study included 195 RP patients, predominantly female (86%) and White (89%), with a mean age of 49 years.
• All patients exhibited ear, nose, or airway involvement, and 83% had musculoskeletal manifestations. The median number of clinical manifestations was 11.
• Organ damage was observed in 41% of patients. Patients treated with JAKis or biologic IS drugs (group 3) were more likely to experience organ damage, arthritis, and subglottic stenosis compared to other groups.

Conclusions:

• Relapsing polychondritis imposes a substantial clinical burden and leads to significant organ damage.
• While GCs are commonly used, the application of other immunosuppressive therapies varies.
• The absence of a standardized treatment approach emphasizes the critical need for further clinical trials and the development of evidence-based treatment guidelines for RP.