Relapses in Neurosarcoidosis: Analysis of Disease Recurrence from a Single Center Cohort
Aditi Sharma1, Ka-Ho Wong1, Tammy Smith1, Melissa Wright1, Jennifer Lord2, M. Paz Soldan1, Robert Kadish1, Jonathan Galli1, John Rose1, Stacey Clardy1
1University of Utah, 2Novant Health
Objective:
To analyze disease relapses within a well-characterized neurosarcoidosis cohort, with particular attention to length of illness, other organ system involvement, treatment strategy and duration, lesion location, and outcomes in patients with neurosarcoidosis within the University of Utah Healthcare System
Background:
Neurosarcoidosis is notable for variable clinical manifestations and outcomes, as well as lack of FDA-approved treatments or high-quality prospective trials to guide treatment. We do not have evidence-based guidelines for immunotherapy selection or treatment duration.
Design/Methods:
Inclusion criteria for our cohort included: (i) At least one ICD-9-CM 135 or ICD-10-CM D86 (sarcoidosis) and (ii) At least 3 outpatient visits with a University of Utah clinician in the Neurology Department within the University of Utah electronic health record between July 1, 2010, through September 30, 2023. Neurosarcoidosis diagnoses were confirmed via a neuroimmunologist review of individual patient charts.
Results:
78 patients met criteria for inclusion in the cohort. The average age of the cohort was 57 years, with 62% female. We analyzed: (i) Presenting neurologic and systemic symptoms (ii) Serial imaging and disease activity in relation to treatment, with corresponding clinical features and other organ involvement (iii) Relapse rate and timing with a special emphasis on duration and dose of corticosteroids (iv) Maintenance therapy and eventual functional outcomes.
Conclusions:
Relapses in neurosarcoidosis are challenging to predict, and the prevention of relapse must be balanced with risk of prolonged immunosuppression and individual patient comorbidities. Data is lacking regarding optimal immunotherapy selection, as well as the timing of de-escalation or cessation of immunotherapy once stability is attained in neurosarcoidosis patients.