Background/Purpose: We conducted a retrospective survey study in 2009 and collected clinical data of 239 patients with relapsing polychondritis (RP). Using the survey data, we found a strong inverse relationship between ear and airway involvement, suggesting that the two major clinical symptoms of RP occurred in a mutually exclusive manner (Arthritis Rheumatol. 2018). When we divided the patients into three subgroups, namely patients with airway involvement (A subgroup, 20% of 239 patients), patients with ear involvement (E subgroup, 49%), and patients with both ear and airway involvement (B subgroup, 29%), patients in B subgroup had more progressive and longer disease course (Medicine. 2018). Actually, 59% patients presented with ear involvement at disease onset and a third of the patients were allocated into B subgroup (patients with both ear and airway involvement) with a high mortality rate (BMC Rheumatol. 2020). To confirm the issue, we conducted 2nd nationwide survey in 2019.

Methods: The survey questionnaire was sent to Japanese major medical facilities and outpatient clinics in 2019. In this study, we analyzed clinical data of 128 patients with RP of the 2nd survey and made comparison with those of 239 patients with RP of the 1st survey.

Results: In 2019 survey, patient age, disease duration, and male to female ratio were 60.7 ± 1.36 years (57.4 ± 1.07 years in the 1st survey conducted 2009), 8.26 ± 0.74 years (4.76 ± 0.33 years), and 1.13:1 (1.13:1), respectively. The disease duration in 2019 survey was significantly longer than those in 2009 survey (p < 0.001). Numbers of the involved organs per-patient basis of 2019 survey were 1.30 ± 0.06 organs at disease onset and 3.09 ± 0.13 organs at the last follow-up (8.26 years). In 2009 survey, numbers of the involved organs at onset were 1.13 ± 0.02 organs and increased to 3.13 ± 0.09 organs at the last (4.76 years). The comparison suggests that the disease progressed modestly taking more time in 2019 survey. The prognostic stages tended to improve (from 2.45 ± 0.07 to 2.21 ± 0.07, p = 0.06) and the mortality rate declined significantly (from 9.2% to 2.3%, p = 0.01) in the 2019 survey compared with 2009 survey. At the last follow-up, incidence of ear (79% versus 89%, p = 0.02) and joint involvement (39% vs. 57%, p = 0.001) was significantly higher in 2019 survey than that in 2009 survey. Incidence of airway (49% vs. 28%, p = 0.001) and CNS (12% vs. 4.7%, p = 0.03) involvement was significantly lower in 2019 survey than that in 2009 survey. Use of immunosuppressants (except steroids) was significantly frequent in 2019 survey (59.4 ± 4.36%) than that in 2009 survey (37.2 ± 3.13%, p < 0.001).

Conclusion: In patients with RP, incidence of airway involvement and their mortality rate declined significantly during the decade in Japan. We are hard to exclude a possibility that RP spontaneously became mild in the decade. Nonetheless, our second survey suggests important implications of current treatment and/or management of RP, including advances of medications.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-nation-wide-epidemical-study-on-2009-and-that-on-2019-revealed-that-improvement-of-disease-severity-and-mortality-rate-may-come-from-progress-of-proficient-management-in-patients-with-re/