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

Orphanet Journal of Rare Diseases

Shuang Yun¹  Shitao Zhang²  Rui Yang³  Xiaojun Liu⁴  Tiange Wu⁴  Shengyun Liu⁴  Tianfang Li⁴  Lei Zhang⁴  Jinyan Guo⁴  Lishuai Han⁴  Jiameng Lu⁴  Yujie He⁴ 

[1] Ophthalmology Department, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China;Otolaryngology Department, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China;Pulmonology Department, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China;Rheumatology Department, The First Affiliated Hospital of Zhengzhou University, E1 Jianshe Road, 450052, Zhengzhou, Henan, China;

关键词: Relapsing polychondritis;    RPC;    Classification;    Clinical patterns;    Diagnosis;   

DOI  :  10.1186/s13023-021-01861-x

来源: Springer

【 摘 要 】

BackgroundRelapsing polychondritis(RPC) is a rare autoimmune disease and its early diagnosis remains challenging. Defining the clinical patterns and disease course may help early recognition of RPC.ResultsSixty-six males and 60 females were included in this study. The average age at onset were 47.1 ± 13.8 years and the median follow-up period was 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, a minority of limited RPC patients progressed into systemic form. Patients with Ear pattern had the highest survival rate and relatively 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. The clinical patterns and presenting forms may evolve during follow-up. Our findings may facilitate early recognition of this rare disease.

【 授权许可】

CC BY