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

Abstract

Objective: Relapsing polychondritis (RP) is a rare, heterogeneous, multisystem disease lacking standard treatment guidelines. This study describes clinical manifestations in association with approaches to treatment.

Methods: Adults with physician-diagnosed RP were recruited into a multicenter observational cohort study. Clinical manifestations, organ damage, and medication history were recorded at the baseline study visit. Treatments received for RP at any time point before the initial visit were categorized into three groups: group 1 was treated with glucocorticoids (GCs) or no drugs, group 2 was treated with nonbiologic immunosuppressive (IS) drugs excluding JAK inhibitors (JAKis) with or without GCs, and group 3 was treated with JAKis or biologic IS drugs with or without nonbiologic IS drugs or GCs.

Results: Included in the study were 195 patients with RP who were predominantly female (167, 86%) and White (174, 89%), with a mean age of 49 ± 13years. All patients had ear, nose, or airway involvement, and 163 (83%) had musculoskeletal manifestations of RP. All patients had at least three clinical manifestations with median of 11 (range 3-19). GC treatment was given to 186 (95%) patients. Organ damage was seen in 80 (41%) patients. Treatment groups 1, 2, and 3 had 37 (19%), 55 (28%), and 103 (53%) patients, respectively. Patients in group 3 were more likely to have organ damage, arthritis, and subglottic stenosis.

Conclusion: Patients with RP have a high burden of clinical manifestations with resultant damage. Physicians typically treat RP with GCs, and the use of other immunosuppressive medications is variable. Absence of a consensus approach to treatment underscores the need for clinical trials and treatment guidelines for RP.

Figure 1

Clinical manifestations of relapsing polychondritis. (A) auricular chondritis, (B) auricular deformity, (C) nasal chondritis, (D) saddle nose deformity, (E) tracheal stenosis, (F) tracheal thickening; (G) tracheal and bronchial wall calcification. The red arrows show (F) areas of tracheal thickening and (G) tracheal and bronchial wall calcifications.

Figure 2

Different immunomodulatory medications used in patients with relapsing polychondritis in the study cohort. JAKi, JAK inhibitor; TNF, tumor necrosis factor.