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Diagnosing relapsing polychondritis remains a common challenge: experience from a Chinese retrospective cohort

Abstract

Objective

The diagnosis of relapsing polychondritis (RP) is often mistaken or delayed. In this retrospective cohort, we aimed to unveil the causes responsible for such phenomenon, to determine the associated factors, and to compare diagnosis in clinical settings with the current diagnostic criteria.

Method

Eighty-seven RP patients followed-up by rheumatologists from January 1, 2008, to October 31, 2018, were retrospectively analyzed.

Results

A total of 50 male and 37 female patients were included with a mean age of 45.9 ± 14.5 years. Ninety-three percent were initially admitted by non-rheumatologic specialists .Twenty-eight percent were correctly diagnosed, while 72% were misdiagnosed at the first visits, all by non-rheumatologic specialists. Patients admitted by non-rheumatologic specialists had increased odds of misdiagnosis (odds ratio [OR] = 1.3, 95% confidence interval [95% CI] 1.1–1.7, P = 0.000). Fifty-seven (65.5%) patients did not meet with Michet or Damiani criteria, with 16 (18.4%) patients diagnosed as partial RP and 41( 47.1%) patients diagnosed as limited RP.

Conclusions

Incorrect and delayed diagnosis of RP is common in our cohort, and insufficient awareness of the disease in non-rheumatologic specialists at least partially contributes to this. It is imperative to revise the current criteria for early diagnosis.

Key Points
Diagnosing relapsing polychondritis (RP) in early stage remains challenging after all these years, especially among non-rheumatologic specialists, indicating the importance of teaching non-rheumatologic specialists to improve their understanding of this rare disease.
Many RP patients did not fully meet with the current criteria, suggesting that revision of the current criteria is imperative for early diagnosis of this rare disease.

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Data availability

Data sharing not applicable to this article as no datasets were generated or analyzed during the current study.

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Funding

The authors received no financial and material support.

Author information

Correspondence to Sheng-Yun Liu or Tian-Fang Li.

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Ethical approval

This retrospective study was approved by the Ethic Committee of Zhengzhou University (SR-2018-LW-050) and was conducted in accordance with the Declaration of Helsinki. When analyzing the data, personal information such as name, hospital number, etc. were replaced by index numbers.

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Cite this article

Zhang, L., Wu, T., He, Y. et al. Diagnosing relapsing polychondritis remains a common challenge: experience from a Chinese retrospective cohort. Clin Rheumatol (2020). https://doi.org/10.1007/s10067-019-04852-3

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Keywords

  • Clinical features
  • Criteria
  • Delay
  • Misdiagnosis
  • Relapsing polychondritis