ABSTRACT
Objective:To explore the clinical profile and prognostic features of relapsing polychondritis (RP) with nervous system involvement.Methods:The clinical data of 28 RP patients with nervous system involvement admitted to Peking Union Medical College Hospital from December 2005 to November 2019 were collected and analyzed.Results:The median age of the patients was 50 years and 57.1% (16/28) were men. Patients with central nervous system involvement accounted for 92.9% (26/28) of the total. Common manifestations included headache (69.2%, 18/26), cognitive impairment (38.5%, 10/26), and mental disorders (38.5%, 10/26); only two cases had peripheral nervous system damage. Brain magnetic resonance imaging revealed that subcortical (13/18) and cortical (8/18) tissues were most affected. Cerebrospinal fluid tests showed that the median white blood cell count was 55×10 6/L, while the protein level was also slighted elevated ((0.54±0.17) g/L). All patients were treated with glucocorticoids, and 85.7% (24/28) of patients with immunosuppressants; 27 patients showed optimistic responses to the treatment, while 24 had recurrent courses. Cognitive dysfunction was the most common residual symptom. Follow-up data of 16 patients were available with an average follow-up time of 3.3 years. Ten of the patients were in stable condition and three patients died. Conclusions:Nervous system damage, especially limbic encephalitis and meningitis may present in course of RP. However, it is a challenge to diagnose for the non-specific clinical manifestations. Treatment with corticosteroids and immunosuppressants can lead to a favorable response.