Abstract

We conducted retrospective cohort studies of patients with relapsing polychondritis (RP) twice in 2009 and 2019, using a physician questionnaire. We compared the patients’ clinical statuses between the years. Age and gender were comparable between the two surveys. Mean disease duration was longer in 2019 survey (8.3 years) than that in 2009 survey (4.8 years, P < 0.001). The mortality rate declined in 2019 survey compared with those in 2009 survey (from 9.2 to 1.6%, P < 0.001). Incidence of airway involvement decreased in 2019 survey compared with that in 2009 survey (from 49 to 37%, P = 0.012). In 2019 survey, we found more frequent use of biological agents and immunosuppressants in patients with airway involvement. When we focused on RP patients with airway involvement, physicians in 2019 chose methotrexate and calcineurin inhibitors preferentially, compared with azathioprine and cyclophosphamide. Of note is that increased use of infliximab was observed in RP patients with airway involvement, but not in those without. Reduction of airway involvement and mortality in patients with RP was observed in 2019 survey. The reduction may associate with the frequent use of biologics including infliximab in RP patients with airway involvement.

Highlights

  • We conducted retrospective cohort studies of patients with relapsing polychondritis (RP) twice in 2009 and 2019, using a physician questionnaire

  • We collected newly anonymous information of 190 RP patients in 2019 survey, and the demographic and clinical information were compared with 239 anonymous patient data of 2009 survey (Table 1)

  • Numbers of RP patients treated with tracheotomy and/or stenting decreased in 2019 survey (P < 0.001, Table 1)

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Summary

Introduction

We conducted retrospective cohort studies of patients with relapsing polychondritis (RP) twice in 2009 and 2019, using a physician questionnaire. In 2019 survey, we found more frequent use of biological agents and immunosuppressants in patients with airway involvement. Reduction of airway involvement and mortality in patients with RP was observed in 2019 survey. The reduction may associate with the frequent use of biologics including infliximab in RP patients with airway involvement. We conducted an epidemiological survey of RP patients in 2009 (termed as 2009 survey) using a physician questionnaire and collected the clinical information of 239 ­patients[5]. A recent study demonstrated that TNF inhibition improved clinical and laboratory parameters, such as respiratory symptoms and results of pulmonary function test, in RP patients with airway i­nvolvement[7]. Recent studies revealed that 10-year and 8-year survival rates were 91%4 and 94%6, respectively

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