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Abstract：
Relapsing 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.
Sixty-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.
RPC 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.
Sixty-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.
RPC 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.
摘要：
复发性多软骨炎（RPC）是一种罕见的自身免疫性疾病，其早期诊断仍然具有挑战性。定义临床模式和病程可能有助于早期识别RPC。
本研究包括66名男性和60名女性。平均发病年龄为47.1±13.8岁，中位随访时间为18个月。相关分析显示气道受累与耳廓软骨炎呈显著负相关(r=-0.75,P<0.001)。确定了四种不同的临床模式：耳朵模式（50.8%），气道模式（38.9%），重叠模式(4.8%)和气道-耳负模式(5.6%)，耳朵模式和气道模式的患者进一步分为有限形式和全身形式的RPC(27.8%的耳朵模式有限形式和24.6%的初始气道模式有限形式)。随访期间,少数耳朵模式和气道模式的患者进展为重叠模式，一些气道耳部阴性模式患者进展为耳部模式。虽然大多数有限的RPC患者在随访期间仍然是有限的形式，少数有限的RPC患者进展为全身形式。Ear型患者的生存率最高，炎症状态相对较低。
RPC患者可根据器官受累分为4种不同的临床模式和2种不同的表现形式（有限和全身性）。临床模式和呈现形式可能在随访期间演变。我们的发现可能有助于早期识别这种罕见疾病。
本研究包括66名男性和60名女性。平均发病年龄为47.1±13.8岁，中位随访时间为18个月。相关分析显示气道受累与耳廓软骨炎呈显著负相关(r=-0.75,P<0.001)。确定了四种不同的临床模式：耳朵模式（50.8%），气道模式（38.9%），重叠模式(4.8%)和气道-耳负模式(5.6%)，耳朵模式和气道模式的患者进一步分为有限形式和全身形式的RPC(27.8%的耳朵模式有限形式和24.6%的初始气道模式有限形式)。随访期间,少数耳朵模式和气道模式的患者进展为重叠模式，一些气道耳部阴性模式患者进展为耳部模式。虽然大多数有限的RPC患者在随访期间仍然是有限的形式，少数有限的RPC患者进展为全身形式。Ear型患者的生存率最高，炎症状态相对较低。
RPC患者可根据器官受累分为4种不同的临床模式和2种不同的表现形式（有限和全身性）。临床模式和呈现形式可能在随访期间演变。我们的发现可能有助于早期识别这种罕见疾病。
