CNS involvement in a patient with primary Sjögren’s syndrome: a case report

Dr Kathi Ging at the Institute of Anatomy, University of Zurich, Zurich, Switzerland and colleagues have reported a rare case of CNS involvement in a patient with primary Sjögren’s syndrome. The case has appeared in the Journal of Medical Case Reports.

Sjögren syndrome is a chronic systemic autoimmune disease affecting 2–3% of adults. It is classified as primary when occurring in isolation and as secondary when associated with another autoimmune disease. Primary Sjögren syndrome is characterized by mononuclear infiltration and destruction of the exocrine glands, mainly the lachrymal and salivary glands, resulting in xerophthalmia and xerostomia

Primary Sjögren’s syndrome is the second most common rheumatological disorder after rheumatoid arthritis. It typically presents as xerophthalmia and xerostomia in postmenopausal women. Involvement of the central nervous system has been recognized, although its pathogenesis and characteristics are poorly understood. Central nervous system complications are a diagnostic challenge and emphasize the need for systematic screening of patients with new peripheral and central neurological symptoms.

The authors report a case of a 58-year-old Swiss woman presenting with rapidly progressive sensorimotor distal polyneuropathy together with new-onset generalized seizures. Initial magnetic resonance imaging (MRI) of the brain performed after the first seizure showed multiple, bihemispheric, confluent white matter hyperintensities with contrast enhancement. Follow-up imaging 3 days after the initial magnetic resonance imaging demonstrated a fulminant disease progression associated with the serious clinical deterioration of the patient. In light of the results of a minor salivary gland biopsy, autoantibody testing, nerve conduction studies, and cranial magnetic resonance imaging, primary Sjögren’s syndrome with cryoglobulinemia type II was diagnosed. Response to plasmapheresis and subsequent administration of cyclophosphamide was favorable.

Even though exocrinopathy is the hallmark of Sjögren’s syndrome, systemic symptoms are observed in one-third of patients. There is an urgent need to better characterize the mechanisms underlying different disease phenotypes and to perform randomized controlled trials in order to provide tailored and evidence-based treatment for primary Sjögren’s syndrome.