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

Zhang, Lei; Wu, Tiange; He, Yayi; Guo, Jin; Han, Ling; Lu, Jiameng; Liu, Shengyun; Li, Tian-Fang

doi:10.1007/s10067-019-04852-3

Cited by 12 publications

(22 citation statements)

References 24 publications

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“…Meanwhile, partial RPC has been reported by Mathew et al [11] as well as in our previous study [6]. Emerging data suggest that limited RPC are not uncommon in clinical practice [6,[17][18][19][20][21][22][23][24].…”

Section: Introductionsupporting

confidence: 66%

“…However, early and prompt diagnosis is di cult to establish because of the inadequacy of clinical manifestations [8]. Meanwhile, partial RPC has been reported by Mathew et al [11] as well as in our previous study [6]. Emerging data suggest that limited RPC are not uncommon in clinical practice [6,[17][18][19][20][21][22][23][24].…”

Section: Introductionmentioning

confidence: 49%

“…Patients that underwent recurrent chondritis associated with deformity, vestibular dysfunction, ocular in ammation, or in ammatory arthritis were diagnosed as partial RPC as suggested by Mathew et al [11]. Limited RPC can also be diagnosed if patients presented with recurrent in ammatory episodes at isolated cartilaginous sites after exclusion of other possible causes and are responsive to glucocorticoids, based on the observations by other groups and our own [6,8,13,[17][18][19][20][21][22][23][24] . The diagnostic criteria used in present study were listed in table 4.…”

Section: Study Populationmentioning

confidence: 96%

“…The incidence of RPC is about 3.5 per million per year in the U.S. [4]and 0.71 per million population per year between 1990 and 2012 in the UK [5]. Often time, patients' rst visits are to non-rheumatologic specialists [5][6][7][8], due to protean manifestations and wax and wane disease courses of RPC, which may lead to misdiagnosis and delayed diagnosis [5][6][7][8][9][10][11][12][13].…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, lack of vigilance to RPC, particularly in non-rheumatologic specialists, may contributes to diagnostic delay and misdiagnosis [6,25,26]. Thus, the analysis clinical patterns of RPC patients, particularly those in early stage as well as partial and limited RPC, may help non-rheumatologic specialists get a clear and complete scenario of this rare disease, facilitating early recognition.…”

Section: Introductionmentioning

confidence: 99%

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Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study

Zhang

Yun

et al. 2020

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Background Relapsing polychondritis(RPC) is a rare autoimmune disease and its early diagnosis pose a challenge to medical committee. Defining the clinical patterns and disease course may help early recognition of RPC. Results Sixty-six males and 60 females were included in this study. Their average age at onset were 47.1±13.8 years with a median follow-up of 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r=-0.75，P<0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%), and patients with Ear pattern and Airway pattern were further divided into limited and systemic form of RPC (27.8%with limited form of Ear pattern and 24.6% with limited form of Airway pattern initially). During follow-up, a minority of patients with Ear pattern and Airway pattern progressed into Overlap pattern, and some Airway-Ear negative pattern patients progressed into Ear pattern. While a large majority of limited RPC patients remained limited form during follow-up, some patients did become systemic form. Patients with Ear pattern had the highest survival rate and lower inflammatory status. Conclusions RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement, and clinical patterns and presenting forms can evolve during follow-up. Our findings may facilitate early recognition of this rare disease.

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