Bortezomib-Responsive Refractory Anti-N-Methyl-d-Aspartate Receptor Encephalitis

A multitude of environmental factors can result in breakdown of immune tolerance in susceptible hosts. Infectious pathogens are among the most important environmental triggers in the pathogenesis of autoimmunity. Certain autoimmune disorders have a strong association with specific infections. Several neurological autoimmune disorders are thought to occur through post-infectious mechanisms. In this review, we discuss the proposed mechanisms underlying pathogen-induced autoimmunity, and highlight the clinical presentation and treatment of several post-infectious autoimmune neurological disorders. We also highlight post-infectious neurological disorders in the setting of recent outbreaks.

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“…151 Tocilizumab and bortezomib have also been reported to be beneficial in refractory cases. 172,173…”

Section: Treatment Considerationsmentioning

confidence: 99%

Post-infectious neurological disorders

Blackburn

Wang

2020

Ther Adv Neurol Disord

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“…Bortezomib is a 26‐S proteasome inhibitor used in the management of plasma cell dyscrasias such as multiple myeloma 9 . Although some evidence exists regarding the role in refractory autoimmune encephalitis, it did not benefit our patient 10 . A point of particular concern in our patient was the cytopenias that required additional therapy and also precluded timely administration of aggressive immunomodulatory therapy.…”

Section: Discussionmentioning

confidence: 84%

Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis

et al. 2021

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A 14‐year‐old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection‐Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the refractory nature of the seizures. The course of the illness was rather stormy, laced with various medical problems viz. hepatic dysfunction, sepsis, hemodynamic, and hematological abnormalities which posed several challenges in the management. Hemophagocytic lymphocytic histiocytosis (HLH) was identified as the etiology of the illness and was treated but without success. The case report highlights the several immunomodulatory strategies that were employed to treat the disease, despite which the outcome was unfavorable.

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“…56 Bortezomib in autoimmune neurologic diseases Seventeen cases of refractory anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis have been described, all, but one, adults (Table 5). [57][58][59][60][61][62][63][64][65] Bortezomib was administered as single agent or in combination with IV/intrathecal rituximab, tocilizumab and cyclophosphamide. All patients responded, and in four cases central nervous system, plasma cell concentration and antibody declined.…”

Section: Tablementioning

confidence: 99%

Bortezomib in autoimmune hemolytic anemia and beyond

Pasquale

Giannotta

Barcellini

et al. 2021

Therapeutic Advances in Hematology

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Bortezomib is a first-in-class, potent, selective and reversible proteasome inhibitor approved for the treatment of multiple myeloma (MM) and relapsed/refractory mantle cell lymphoma. In these diseases, bortezomib targets plasma cells and lymphocytes reducing tumor burden. Recently, preclinical evidence highlighted its efficacy in reducing long-lived plasma cells responsible of autoantibodies production in several models of autoimmune conditions. These findings paved the way to a number of experiences of bortezomib use in patients with various autoimmune conditions, including autoimmune hemolytic anemia (AIHA). The latter is a nice model of autoimmunity in hematology and is caused by the production of autoantibodies against erythrocytes resulting in various degrees of hemolytic anemia. AIHA is classified in warm and cold forms according to the thermal characteristics of the autoantibody, and first-line treatment mainly relies on steroids for warm cases and the anti-CD20 rituximab for cold ones. Relapsed/refractory cases are still an unmet need, and bortezomib has been proposed in this setting with intriguing efficacy. In this review, we collected available literature on bortezomib use in AIHA and in other immune-mediated hematologic and non-hematologic diseases. Overall, most experiences highlight bortezomib efficacy even in multi-relapsed/refractory patients and suggest to consider its use in AIHA after rituximab failure.

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Bortezomib-Responsive Refractory Anti-N-Methyl-d-Aspartate Receptor Encephalitis

Cited by 24 publications

References 17 publications

Post-infectious neurological disorders

Post-infectious neurological disorders

Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis

Bortezomib in autoimmune hemolytic anemia and beyond

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