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J Immunother Cancer. 2018 Oct 22;6(1):110. doi: 10.1186/s40425-018-0429-4.

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

Author information

1
Department of Medicine, Division of Immunology and Allergy, Lausanne University Hospital CHUV, Rue du Bugnon 46, CH-1011, Lausanne, Switzerland.
2
Department of Neurology, Lausanne University Hospital CHUV, Rue du Bugnon 46, CH-1011, Lausanne, Switzerland.
3
Department of Medical Oncology, Lausanne University Hospital CHUV, Rue du Bugnon 46, CH-1011, Lausanne, Switzerland.
4
Ludwig Institute for Cancer Research, Chemin des Boveresses 155, CH-1066, Epalinges, Switzerland.
5
Department of Medicine, Division of Immunology and Allergy, Lausanne University Hospital CHUV, Rue du Bugnon 46, CH-1011, Lausanne, Switzerland. michel.obeid@chuv.ch.
6
Vaccination and Immunotherapy Center, Lausanne University Hospital CHUV, Rue du Bugnon 17, CH-1011, Lausanne, Switzerland. michel.obeid@chuv.ch.
7
Medical School Pitié-Salpêtrière, Sorbonne University, 91 Boulevard de l'Hôpital, F-75013, Paris, France. michel.obeid@chuv.ch.

Abstract

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.

KEYWORDS:

Checkpoint inhibitors; Immune-related adverse events; Neuro-Sjögren’s syndrome; PD-1; Pembrolizumab

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