XXXX ( @entity159 )--The potential role for immunotherapy.

@entity1 @entity410 @entity9372 @entity3391 @entity302 @entity367 @entity230 @entity159
@entity159 ( @entity159 ) is defined as a state of persistent @entity410 with no identifiable etiology in @entity1 without preexisting @entity302 that lasts longer than 24h despite optimal therapy. Management of @entity159 is challenging, and the role of immunotherapy (IT) is unclear. We identified @entity1 fulfilling the criteria for @entity159 at a single institution. These @entity1 were described, analyzed, and compared with @entity159 cases available from the literature. Finally, a pooled analysis of available case series was conducted to compare the outcomes in @entity1 who received IT with those not treated with IT during the course of @entity159 in order to generate hypotheses for further research. In our case series, @entity159 was diagnosed in 11 @entity1 (9 females) with a mean age of 48 years and a mean duration of 54.4 days. Autoantibodies were identified in 7 @entity1 , of which anti- @entity9372 ( @entity367 decarboxylase) and anti-NMDAR ( @entity3391 receptor) were most frequent. Of the 11 @entity1 , 8 were treated with IT (intravenous @entity230 , immunoglobulins, plasmapheresis, or a combination), and 4 received chemotherapy. Of the 8 @entity1 treated with IT, 6 had favorable outcomes (defined as any outcome other than death, vegetative state, or inability to take care of oneself) compared with 0 out of 3 @entity1 who did not receive IT. Difference in outcomes was significant (p=0.026). Pooled analysis of all identified case series, including ours, showed a statistically significant effect (p=0.022), with favorable outcomes in 42% of the @entity1 who received any IT compared with 20% in those who did not. In all @entity1 with refractory SE and negative comprehensive investigations, a diagnosis of @entity159 should be considered. This would aid planning for early immunotherapy. Currently, only Class IV evidence for the use of immunotherapy in @entity159 is available. Prospective multicenter studies are necessary to assess the true efficacy of IT in @entity159 .
