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Central Nervous System Sarcoidosis: A National-Based registry study
Yazan Al-Zamer1, Sami Al-Horani1, Marah Akhdar1
1Jordan University of Science and Technology.
Summary
Central nervous system (CNS) sarcoidosis affects over 11% of patients, predominantly females and Caucasians. This condition often leads to multiorgan involvement, chronic pain, and disability, necessitating corticosteroid treatment.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Sarcoidosis of the central nervous system (CNS) is a rare condition with limited data on its demographics, diagnosis, and treatment.
- Existing literature on CNS sarcoidosis is scarce and often inconsistent, highlighting the need for comprehensive studies.
Purpose of the Study:
- To investigate the demographics, diagnostic methods, management strategies, and clinical complications of CNS sarcoidosis in the United States.
- To provide insights into the characteristics of patients with CNS sarcoidosis using a nationwide registry.
Main Methods:
- A retrospective analysis of 3,835 respondents to the Foundation for Sarcoidosis Research Sarcoidosis Advanced Registry for Cures Questionnaire (FSR-SARC) from June 2014 to August 2019.
- Propensity score matching, summary, univariate, and multivariate analyses were employed to compare patients with and without CNS sarcoidosis.
Main Results:
- 11.55% of patients (420/3634) had CNS sarcoidosis, with a higher prevalence in females (70.5%) and Caucasians (73.1%).
- The mean age at diagnosis was 43.3 years, and 75.7% experienced multiorgan involvement (≥3 organs).
- Cranial MRI was the most common diagnostic tool (76.5% abnormal), and corticosteroids were the primary treatment (49.0%), followed by cytotoxic agents (42.9%).
Conclusions:
- The prevalence of CNS sarcoidosis in this cohort aligns with previous findings.
- Cranial MRI is the most utilized imaging modality for diagnosing CNS sarcoidosis.
- Corticosteroids are the mainstay treatment, and CNS sarcoidosis is associated with significant complications like chronic pain and disability.
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