When the Body Hardens and the Mind Fragments: Psychosis in Systemic Sclerosis

Systemic sclerosis is a chronic autoimmune disease primarily characterized by fibrosis and vasculopathy; neurological involvement is considered uncommon. We present the case of a 27-year-old woman with a history of asthma, Raynaud's phenomenon, and chronic facial dermatosis, who developed an acute psychotic syndrome characterized by hallucinations, disorganized speech, and persecutory delusions, with no prior psychiatric history. Physical examination revealed mucocutaneous findings consistent with systemic sclerosis, and neurological evaluation showed altered cognition and behavioral disturbances. Laboratory and cerebrospinal fluid analyses were unremarkable, including negative anti-NMDA receptor antibodies. Transcranial Doppler suggested increased cerebral vascular resistance, raising suspicion of CNS vasculitis. The patient fulfilled the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis despite negative specific autoantibodies. Neuropsychiatric symptoms persisted despite antipsychotic therapy, but improved markedly following high-dose intravenous methylprednisolone and cyclophosphamide. This case illustrates a rare and atypical neuropsychiatric manifestation of systemic sclerosis, highlighting the importance of recognizing autoimmune contributions to acute psychosis. Timely immunosuppressive treatment can lead to favorable outcomes even in the absence of definitive serological markers. Further studies are needed to elucidate the pathophysiology of central nervous system involvement in systemic sclerosis and to guide diagnostic and therapeutic strategies for such presentations.