RT Journal Article
SR Electronic
T1 A severe case of neuro-Sjögren’s syndrome induced by pembrolizumab
JF Journal for ImmunoTherapy of Cancer
JO J Immunother Cancer
FD BMJ Publishing Group Ltd
SP 110
DO 10.1186/s40425-018-0429-4
VO 6
IS 1
A1 Ghosn, Jaqueline
A1 Vicino, Alex
A1 Michielin, Olivier
A1 Coukos, George
A1 Kuntzer, Thierry
A1 Obeid, Michel
YR 2018
UL http://jitc.bmj.com/content/6/1/110.abstract
AB 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.Jaqueline Ghosn and Alex Vicino contributed equally to this work.Abbreviations:AITAutoimmune toxicityASGBAccessory salivary glands biopsyCPIsCheckpoint inhibitorsCSFCerebrospinal fluidCTDConnective tissue diseaseESRErythrocyte sedimentation rateirAEsImmune-related adverse eventsPD-1Programmed death 1 receptorPD-L1Programmed death-ligand 1SNAPsSensory nerve action potentialsSNNSensory neuronopathy