Catatonia and Psychosis as Manifestations of Primary Sjögren’s Syndrome

Primary Sjögren’s syndrome (SS) is a chronic, systemic autoimmune disease, most commonly presenting with sicca symptoms—xerostomia and xerophthalmia. Up to one-half of affected individuals also develop extra-glandular involvement. Early diagnosis is important to prevent further complications. This paper describes an atypical case of SS in a 21-year-old woman who presented first with neuropsychiatric symptoms and fever. She was diagnosed and treated for a presumed psychotic episode. However, because she remained febrile, a thorough investigation resulted in the challenging diagnosis of SS. LEARNING POINTS: Primary Sjögren’s syndrome (SS) may be an under-recognized cause of central nervous system and neuropsychiatric involvement. Neuropsychiatric manifestations of primary SS can be variable. Patients may be treated incorrectly if the diagnosis is not rapidly established, so a clinical history and thorough examination are very important.