Immunosuppressive Therapies May Reduce Relapse in Neurosarcoidosis

Cerebral MRIs
Cerebral MRIs
Although some patients will recover completely after corticosteroid therapy, some will experience multiple relapses and develop related severe functional impairments.

Immunosuppressive therapies may reduce relapse rates in patients with neurosarcoidosis, according to results from a retrospective study published in JAMA Neurology.

A total of 234 patients with neurosarcoidosis were included in this retrospective cohort study to determine predictive factors associated with immunosuppressive therapies and disease relapse.

According to the investigators, mortality was significantly associated with a high expanded Disability Status Scale score, older age (hazard ratio [HR] per 10 years, 1.64; 95% CI, 1.19-2.27; P =.003), and peripheral nervous system involvement (HR, 6.75; 95% CI, 2.31-19.7; P <.001).

The 10-year relapse-free survival rate was estimated at 28% for neurologic relapses (95% CI, 20%-38%) and 14% (95% CI, 9%-22%) for total relapses. Presence of encephalic symptoms correlated with significantly shorter neurologic relapse-free survival (HR, 2.35; 95% CI, 1.44-3.83; P <.001).

History of tobacco use (odds ratio [OR], 3.64; 95% CI, 1.36-9.73; P =.01), elevated expanded Disability Status Scale score at baseline (OR per point, 1.92; 95% CI, 1.55-2.37; P <.001), and encephalic involvement (OR, 3.04; 95% CI, 1.11-8.38; P =.03) correlated with long-term functional impairment.

The risk for disease relapse was significantly lower among patients taking cyclophosphamide (HR, 0.26; 95% CI, 0.11-0.59; P =.001), infliximab (HR, 0.16; 95% CI, 0.02-1.24; P =.08), and methotrexate sodium (HR, 0.47; 95% CI, 0.25-0.87; P =.02) treatments.

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Investigators of this study did not randomize immunosuppressive therapies, which reduces the ability to draw causal associations between administered treatments and relapse rates.

Although future randomized trials using these therapies may be warranted, the investigators believe the “presence of encephalic or peripheral nervous system involvement in neurosarcoidosis should affect the decision to use immunosuppressive therapies to help lower relapse rates” in current practice.


Joubert B, Chapelon-Abric C, Biard L, et al. Association of prognostic factors and immunosuppressive treatment with long-term outcomes in neurosarcoidosis [published online October 9, 2017]. JAMA Neurol. doi:10.1001/jamaneurol.2017.2492