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Observational Study
. 2020 Aug;72(8):1396-1402.
doi: 10.1002/art.41270. Epub 2020 Jul 8.

Defining Clinical Subgroups in Relapsing Polychondritis: A Prospective Observational Cohort Study

Marcela Ferrada  1 , Casey A Rimland  2 , Kaitlin Quinn  1 , Keith Sikora  1 , Jeff Kim  3 , Clint Allen  3 , Arlene Sirajuddin  4 , Wendy Goodspeed  1 , Marcus Chen  4 , Peter C Grayson  1
Affiliations
Observational Study

Defining Clinical Subgroups in Relapsing Polychondritis: A Prospective Observational Cohort Study

Marcela Ferrada et al. Arthritis Rheumatol. 2020 Aug.

Abstract

Objective: Relapsing polychondritis (RP) is a systemic disease. Failure to recognize RP can lead to diagnostic delay and further complications, including death. This study was undertaken to identify clinical patterns in a prospective cohort of patients with RP.

Methods: Patient subgroups were identified using latent class analysis based on 8 clinical variables: saddle-nose deformity, subglottic stenosis, tracheomalacia, bronchomalacia, ear chondritis, tenosynovitis/synovitis, inflammatory eye disease, and audiovestibular disease. Model selection was based on Akaike's information criterion.

Results: Seventy-three patients were included in this study. Patients were classified into 1 of 3 subgroups: type 1 RP (14%), type 2 RP (29%), and type 3 RP (58%). Type 1 RP was characterized by ear chondritis (100%), tracheomalacia (100%), saddle-nose deformity (90%), and subglottic stenosis (80%). These patients had the shortest median time to diagnosis (1 year), highest disease activity, and greatest frequency of admission to the intensive care unit and tracheostomy. Type 2 RP was characterized by tracheomalacia (100%) and bronchomalacia (52%), but no saddle-nose deformity or subglottic stenosis. These patients had the longest median time to diagnosis (10 years) and highest percentage of work disability. Type 3 RP was characterized by tenosynovitis/synovitis (60%) and ear chondritis (55%). There were no significant differences in sex, race, or treatment strategies between the 3 subgroups.

Conclusion: Our findings indicate that there are 3 subgroups of patients with RP, with differences in time to diagnosis, clinical and radiologic characteristics, and disease-related complications. Recognizing a broader spectrum of clinical patterns in RP, beyond cartilaginous involvement of the ear and upper airway, may facilitate more timely diagnosis.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Figure 1A Represent input variables used for latent class analysis modeling which were chosen based on criterion items from McAdams diagnostic criteria for RP. Figure 1B Reprepresent clinical subtypes identified by latent class analysis. The color demonstrate the percentage of organ involvement. The darker the color the higher the percentage of involvement.
See this image and copyright information in PMC

Comment in

  • Reply.
    Ferrada MA, Grayson PC. Ferrada MA, et al. Arthritis Rheumatol. 2020 Dec;72(12):2167-2168. doi: 10.1002/art.41438. Epub 2020 Oct 7. Arthritis Rheumatol. 2020. PMID: 32686916 No abstract available.
  • Early Recognition of Relapsing Polychondritis: Comment on the Article by Ferrada et al.
    Zhang L, Li T, Liu S, Liu X. Zhang L, et al. Arthritis Rheumatol. 2020 Dec;72(12):2166-2167. doi: 10.1002/art.41439. Epub 2020 Oct 11. Arthritis Rheumatol. 2020. PMID: 32686919 No abstract available.

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