A severe case of neuro-Sjögren’s syndrome induced by pembrolizumab

Background The prevalence of connective tissue disease (CTD) induced by immune checkpoint inhibitors (CPIs) in the absence of pre-existing autoimmunity is unknown. Case presentation We report the case of a melanoma patient treated for 8 months with pembrolizumab who developed a subacute ataxic senso...

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Bibliographic Details
Published in:Journal for immunotherapy of cancer Vol. 6; no. 1; pp. 110 - 6
Main Authors: Ghosn, Jaqueline, Vicino, Alex, Michielin, Olivier, Coukos, George, Kuntzer, Thierry, Obeid, Michel
Format: Journal Article
Language:English
Published: London BioMed Central 22.10.2018
BMJ Publishing Group LTD
BMJ Publishing Group
Subjects:
Antibodies
Biopsy
Case Report
Case Reports
Checkpoint inhibitors
Exocrine glands
Hemoglobin
Hepatitis
Immune-related adverse events
Immunology
Immunotherapy
Life Sciences
Medicine
Medicine & Public Health
Melanoma
Metastasis
Monoclonal antibodies
Neuro-Sjögren’s syndrome
Oncology
PD-1
Pembrolizumab
Remission (Medicine)
Targeted cancer therapy
Viruses
Checkpoint inhibitors
Pembrolizumab
Immune-related adverse events
Neuro-Sjögren’s syndrome
PD-1
ISSN:2051-1426, 2051-1426
Online Access:Get full text
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Summary:Background The prevalence of connective tissue disease (CTD) induced by immune checkpoint inhibitors (CPIs) in the absence of pre-existing autoimmunity is unknown. Case presentation We report the case of a melanoma patient treated for 8 months with pembrolizumab who developed a subacute ataxic sensory neuronopathy (SNN), including a right trigeminal neuropathy. Salivary gland biopsy showed inflammatory changes suggestive of Sjögren’s syndrome, while brain MRI revealed enhancement of the right trigeminal ganglia. A high level of protein and pleocytosis was found in the cerebrospinal fluid, with negative cultures. Nerve conduction studies revealed the absence of sensory nerve action potentials in the upper and lower limbs and reduced motor responses in the upper limbs, fulfilling criteria for SNN. Blood tests revealed an important inflammatory syndrome, hemolytic anemia, elevation of total IgG levels and the presence of ANA autoantibodies specific to anti-SSA (52 and 60 kd). All these elements were absent before the initiation of the treatment with pembrolizumab. Initially, there was a clinical response following intravenous frontline methylprednisone, but the subacute relapse required the introduction of second-line treatment with intravenous immunoglobulins and then rituximab, which led to a quick clinical improvement. Conclusions Herein, we describe the first case of a patient who developed a typical SNN as a complication of severe neuro-Sjögren’s syndrome induced by pembrolizumab treatment.
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ISSN:2051-1426
2051-1426
DOI:10.1186/s40425-018-0429-4