News on Relapsing Polychondritis: The Patient's Experience

et al. 2020

Preprint

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.

Section: Discussioncontrasting

confidence: 81%

Section: Study Populationmentioning

confidence: 99%

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

et al. 2020

Preprint

“…Patients younger than 18 years or without complete electronic case files were excluded. Patients with positive anti-neutrophilic cytoplasmic antibody (ANCA) against proteinase-3 (PR3) were also excluded as suggested by Piette and colleagues [30]. The diagnosis was carefully # Sixty-four percent presented symptoms for more than 5 years before diagnosis.…”

Section: Study Populationmentioning

confidence: 99%

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

et al. 2020

Preprint

BackgroundRelapsing polychondritis(RPC) is a rare autoimmune disease, of which the diagnosis in early stage is challenging. Defining the clinical patterns and disease evolution may help early recognition of this rare disease.ResultsSixty-six males and 60 females were included with onset age of 47.1±13.8 years and followed up for a median 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 subdivided as limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern and some Airway-Ear negative pattern patients progressed into Ear pattern. Most of the limited RPC patients remained limited form during follow-up while some patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative lower inflammatory status.ConclusionsRPC 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 and contribute to an updated classification criteria covering all the clinical spectrum of RPC

“…Patients with positive anti-neutrophilic cytoplasmic antibody (ANCA) against proteinase-3 (PR3) were also excluded as suggested by Piette and colleagues [30]. The diagnosis was carefully evaluated and jointly established by a group of rheumatologists and physicians in other specialties.…”

Section: Study Populationmentioning

confidence: 99%

Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: analysis of 126 Chinese patients

et al. 2020

Preprint

Objective To explore the clinical patterns of relapsing polychondritis(RPC) to improve clinicians’ comprehensive understanding of the disease. Methods The electronic records of RPC patients that were hospitalized and followed-up by rheumatologists at our hospital between January 1, 2008 and August 31,2019 were retrospectively analyzed. Results Sixty-six males and 60 femaleswere included with onset age of 47.1±13.8 years and followed up for a median of 18 months. Eighty-seven patients (69.1%) did not met with the traditional criteria.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 subdivided as limited and systemic form of RPC (27.8%with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern andsome Airway-Ear negative patternpatients progressed into Ear pattern. Most of the limited RPC patientsremained limited form during follow-up whilesome patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative lower inflammatory status. Conclusion RPC patients can be categorized as 4 differentclinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement.Limited form of RPC is not uncommon in Chinese patients and traditional criteria of RPC are not suitable for early diagnosis.