Abstract
Objectives
Adult onset Still’s disease (AOSD) is a multifactorial systemic autoinflammatory disease. Neurological damage has been scarcely reported in AOSD. We aimed to characterize the clinical features of AOSD patients with neurological involvement.
Methods
Totally, 187 AOSD patients were admitted to Peking Union Medical College Hospital from January 2015 to August 2019. The complete medical records were reviewed in this retrospective study. Clinical features of 14 AOSD patients with neurological involvement were collected and compared with those without.
Results
The prevalence of neurological involvement in AOSD inpatients was 7.5%. Median disease duration was 4.5 months with a range of 1 to 15 months. The frequent symptoms were fever (14, 100%), rash (13, 92.9%), liver dysfunction (11, 78.6%), arthralgia/arthritis (10, 71.4%), and lymphadenopathy (10, 71.4%). Four (28.6%) patients had macrophage activation syndrome (MAS). Aseptic meningitis was the most common presentation (64.3%) when the nervous system was involved. Other rare manifestations included cranial nerve palsy, encephalitis, and cerebral infarction. Rate of MAS, serum levels of lactate dehydrogenase and ferritin were significantly higher in AOSD patients with neurological involvement than in those without. All patients received high dose corticosteroid therapy and immunosuppressive agents, and two were given tocilizumab. Clinical remission was achieved in all of the 14 AOSD patients with neurological involvement.
Conclusion
Neurological involvement, particularly aseptic meningitis, is not a rare complication of AOSD. It is frequently complicated by MAS. There may be a potential relationship between neurological damages of AOSD and MAS.
Aseptic Meningitis and Cranial Nerve Palsy Revealing Adult‐Onset Still's Disease
