Autoimmune encephalitis (AE) encompasses a spectrum of immune-mediated brain disorders that cause severe neuropsychiatric manifestations. These disorders often lead to protracted hospital stays, complex neurocognitive rehabilitation needs, and significant psychosocial strains on caregivers. Clinical outcomes are better with early recognition and prompt immuno-modulation. Pediatric rheumatologists may encounter children with AE while evaluating for NPSLE or CNS vasculitis, A survey of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) membership was conducted to assess the current roles of pediatric rheumatologists in caring for children with AE.

Methods:

Members of CARRA completed a Web-based survey (N=439 total members; 375 medical providers). 4 emails were sent over a 2 month period (June to August 2015). The study was submitted and exempted from review by Duke University’s Institutional Review Board. 

Results:

101 CARRA voting members (88% in US) responded to the online survey (26% response rate). 74% of respondents evaluated a patient with AE during the past year. Most respondents’ divisions (57%) cared for less than 5 children with AE per year, with 15% of divisions caring for over 10 patients/year. The AE subtypes most frequently encountered were NMDAR encephalitis (84%) and seronegative AE (clinical phenotype without an identified antibody) (80%). All respondents agreed that rheumatologists should rule out traditional rheumatic disorders affecting the CNS, with 60% also acknowledging a role in work up and diagnosis of AE. While 80% recommended a treatment protocol once a diagnosis was made, 20% of respondents stated that rheumatology was the primary service for AE patients. Of the 70 rheumatologists involved in a therapy role, 63% wrote for immunosuppressive medications. Most respondents became involved in care after 1st line therapy was already given (59%). Only 17% of respondents stated that their institutions had a standard protocol for children with AE (mostly for NMDAR). A collaborative relationship between rheumatology and neurology services was present in most locations (85%).Survey comments did reveal challenges in defining the roles of these two services. 32% (21/66) of respondents stated that their institutions had a neuro-immunology service/clinic). 76% of these neuro-immunology services included a pediatric rheumatologist. The most common challenges encountered in the care of children with AE included: inflammatory brain disease not covered in training (58%), lack of curricular development (42%), difficulty collaborating with neurology (41%), and lack of family resources (25%). 27% of respondents did not feel AE was part of rheumatology scope of practice.

Conclusion:

The majority of survey respondents are involved in caring for children with AE. Rheumatologists find work-up and management of AE challenging due to inadequate experience/training, absence of standardized protocols, absence of or challenges in collaboration with neurology and lack of educational resources. Survey results shaped subsequent multi-disciplinary sessions at both 2016 CARRA and ACR meetings that included neurology and rheumatology practitioners.