Manifestations and Treatment in Relapsing Polychondritis

A large multicenter cohort study of Relapsing Polychondritis (RP) describes the clinical manifestations and treatment approaches to this rare, heterogeneous, multisystem disease.

Adults with RP were recruited into a multicenter observational cohort study. Clinical manifestations are described and treatments 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.

A total of 195 RP patients were recruited and were predominantly female (86%) and White (89%), with a mean age of 49 years. 

All had ENT or airway involvement, and 83% had musculoskeletal manifestations. 

The multisystem nature of the disease revealed that all at least three clinical manifestations with median of 11 manifestations (range 3–19). 

Organ damage was seen in 80 (41%) patients. 

GC treatment was given to nearly all (95%) patients. Treatment patterns: 

  • Group 1: GCs or no drugs - 19%, 
  • Group 2: immunosuppressives (+/- GCs) - 28%
  • Group 3: Biologics or JAKi (+/- GCs or IM) -  53%).  
    • Group 3 patients were more likely to have organ damage, arthritis, and subglottic stenosis.

The apparent absence of a consensus on treatment underscores the need for clinical trials and practice guidelines for RP.

Early, accurate diagnosis of RP is essential.  There are proposed diagnostic criteria, each requiring the presence of chondritis in different organ systems or histologic confirmation of chondritis. Yet, there are RP patients with RP who may not fulfill these diagnostic criteria
 
Timely diagnosis leads to more effective therapy and the avoidance of organ damage or life-threatening complications, such as tracheomalacia, bronchomalacia, subglottic stenosis, saddle nose deformity, external ear deformity, and permanent hearing loss.
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Clinical Manifestations and Treatment in Patients With Relapsing Polychondritis: A Multicenter Observational Cohort Study

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Disclosures
The author has no conflicts of interest to disclose related to this subject
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