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Association of prognostic factors and immunosuppressive treatment with long-term outcomes in neurosarcoidosis

JAMA Neurology Oct 26, 2017

Joubert B, et al. - The clinicians performed this retrospective study to identify prognostic factors of and examine the relationship of immunosuppressive treatment with relapse of neurosarcoidosis (NS). This research identified putative factors affecting morbidity and mortality in patients with NS. Among these patients, immunosuppressive therapies (ie, intravenous cyclophosphamide, methotrexate, and infliximab) could be correlated with lower relapse rates.

Methods
  • From January 1, 1990, through December 31, 2015, the clinicians enrolled a cohort of 234 patients fulfilled the diagnostic criteria for NS in a tertiary referral center in Paris, France.
  • In this study, the median follow-up was 8 years (range, 2 months to 23 years).
  • They examined all neurologic and extraneurologic data and treatments.
  • They assessed functional outcomes measured by the absolute value and the variation from baseline of the Expanded Disability Status Scale (EDSS) score at 60 months after the diagnosis, overall survival, and relapse-free survival (RFS).
  • They stratified analyses by the period of NS diagnosis (1990-1999 vs 2000-2015).

Results
  • The clinicians included 234 patients undergoing assessment (117 women (50.0%) and 117 men (50.0%)); median age was 42 years (interquartile range, 32-53 years).
  • The probable 10-year survival rate was 89% (95% CI, 84%-94%).
  • Factors associated with mortality were older age (hazard ratio [HR] per 10 years, 1.64; 95% CI, 1.19-2.27; P = .003), peripheral nervous system involvement (HR, 6.75; 95% CI, 2.31-19.7; P < .001), and higher baseline EDSS score (HR per point, 1.21; 95% CI, 1.06-1.39; P = .005).
  • The estimated 10-year RFS rate for all relapses was 14% (95% CI, 9%-22%) and 28% (95% CI, 20%-38%) for neurologic relapses.
  • In this study, encephalic involvement was correlated with shorter neurologic RFS (HR, 2.35; 95% CI, 1.44-3.83; P < .001).
  • A lower risk for relapse was correlated with cyclophosphamide (HR, 0.26; 95% CI, 0.11-0.59; P = .001), methotrexate sodium (HR, 0.47; 95% CI, 0.25-0.87; P = .02), and infliximab (HR, 0.16; 95% CI, 0.02-1.24; P = .08) treatments.
  • In 160 patients (68.4%), follow-up was greater than 60 months.
  • Factors associated with an EDSS value of at least 2.5 at 60 months were an elevated baseline EDSS score (odds ratio [OR] per point, 1.92; 95% CI, 1.55-2.37; P < .001), tobacco use (OR, 3.64; 95% CI, 1.36-9.73; P = .01), encephalic symptoms (OR, 3.04; 95% CI, 1.11-8.38; P = .03), and less than 4 extraneurologic sarcoidosis localizations (OR, 3.06; 95% CI, 1.04-8.98; P = .04).
  • At 60 months, encephalic involvement (16 of 17 patients [94.1%]; P = .008) and peripheral nervous system involvement (5 of 17 patients [29.4%]; P = .03) were associated with worsening of the EDSS score.
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