Orbital Inflammatory Disease as Initial Presentation of Primary Sjögren Syndrome

Sjögren syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland functions. It also has been associated with musculoskeletal, dermatologic, respiratory, cardiac, nervous system, renal, gastrointestinal, and hematologic involvement. Neurologic involvement, when present, is more often pure sensory neuropathy. Orbital inflammation with abducens nerve (cranial nerve VI) palsy is a previously unreported complication of Sjögren syndrome (SS). A 61-year-old woman who presented with diplopia on lateral gaze, eye pain, and periorbital swelling was diagnosed with orbital inflammation and primary SS. She was treated successfully with high-dose steroids and cyclophosphamide.