Consensus Recommendations for the Management of Neurosarcoidosis: A Delphi Survey of Experts Across the US

ObjectiveTo develop consensus guidelines on the management of neurosarcoidosis via a Delphi survey of expert clinicians across the US. BackgroundNeurosarcoidosis is a rare granulomatous disorder of the nervous system. Expert-consensus diagnostic criteria by the Neurosarcoidosis Consortium Consensus Group provided a framework for diagnosis, but at present there are no guidelines on treatment and surveillance of treatment response in neurosarcoidosis. Design/MethodsAnonymous, Delphi-style surveys of experts were distributed via RedCap. We collected data on the providers’ backgrounds and experience in neurosarcoidosis, and queried respondents on their approach to treatment in general and to specific neurosarcoidosis phenotypes. Consensus was defined as >80% agreement among respondents. ResultsOf 41 invited experts, 32 participated in Survey 1 and 24/32 in Survey 2. All except one were neuroimmunologists. Consensus was reached on the following key questions: initial treatment depends on neurosarcoidosis phenotype and severity; a uniform stepwise escalation approach is not recommended for all phenotypes; glucocorticoids should be administered as part of the initial treatment of neurosarcoidosis regardless of phenotype; active, systemic sarcoidosis should be considered when making treatment decisions; following clinical remission, attempt to wean glucocorticosteroid within the first year of treatment is reasonable; MRI with contrast should be part of treatment response surveillance. Additional consensus recommendations will be presented. ConclusionsExpert consensus can guide practicing clinicians in providing care to those with neurosarcoidosis. Using the Delphi approach, we were able to achieve consensus recommendations for management that could be useful to clinicians in their daily practice. The key recommendation that treatment with higher-efficacy agent should be considered for certain severe phenotypes early on represents a conceptual shift from the traditional ‘escalation’ approach that required patients to fail one or more agents before trying a higher-efficacy drug.