PT  - JOURNAL ARTICLE
AU  - Jaqueline Ghosn
AU  - Alex Vicino
AU  - Olivier Michielin
AU  - George Coukos
AU  - Thierry Kuntzer
AU  - Michel Obeid
TI  - A severe case of neuro-Sjögren’s syndrome induced by pembrolizumab
AID  - 10.1186/s40425-018-0429-4
DP  - 2018 Dec 01
TA  - Journal for ImmunoTherapy of Cancer
PG  - 110
VI  - 6
IP  - 1
4099  - http://jitc.bmj.com/content/6/1/110.short
4100  - http://jitc.bmj.com/content/6/1/110.full
SO  - J Immunother Cancer2018 Dec 01; 6
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