Background/Purpose: We have reported that 239 patients with relapsing polychondritis (RP) were divided 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 airway and ear involvement (B subgroup, 29%) (Medicine. 2018; 97(42): e12837.). Each subgroup exhibited characteristic clinical features. Patients in A subgroup and patients in E subgroup were characterized by saddle nose deformity and central nervous system (CNS) disorders, respectively. Patients in B subgroup had clinical characteristics of progressive and long disease course. We suggested that the disease progressed from A and E subgroups to B subgroup.

Methods: In the current study, we compared organ involvement at disease onset with that at the last follow-up to assess whether the disease progressed from A and E subgroups to B subgroup in patients with RP. We measured serum matrix metalloproteinase-3 (MMP3) concentrations of 32 samples obtained from newly recruited RP patients.

Results: At the first visit, patients had airway involvement (namely A subgroup at disease onset, 18% of 239 patients), ear involvement (E subgroup at disease onset, 56%), both airway and ear involvement (B subgroup at disease onset, 1.7%), eye involvement (7.1%), inner ear dysfunction (3.8%), arthritis (2.9%), CNS involvement (2.5%), nasal chondritis (1.3%), and skin involvement (0.84%). 34% of RP patients in E subgroup at disease onset developed airway involvement by the last follow-up (mean follow-up, 6.0 years) with a significantly higher mortality rate (13%) compared with those without airway involvement (2.3%). Cumulative incidence of CNS involvement was 12% (28 patients) in this study and encephalitis and meningitis were the most frequent manifestations (12 patients, 5.0%). Encephalitis/meningitis occurred before the onset of obvious chondritis in 6 patients (2.5%). In another cohort of RP patients, serum MMP3 concentrations were significantly higher in B subgroup (n=13) than those in A subgroup (n=7) and E subgroup (n=12).

Conclusion: RP patients with disease progression from E subgroup to B subgroup had higher mortality than those remaining in E subgroup at the last follow-up. Caution should be exercised in the diagnosis of CNS involvement in RP patients because of the varying clinical course at disease onset. Although the further study is necessary, MMP3 in RP patients may aggravate the inflammatory response of chondrocytes and promote overlapping organ involvement.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-third-of-relapsing-polychondritis-patients-with-ear-involvement-at-presentation-had-airway-involvement-at-the-last-follow-up-with-a-high-mortality-rate/