Abstract

This article describes the neurologic manifestations of systemic rheumatologic disorders. Although most have historically been classified as autoimmune disorders, rheumatologic diseases are increasingly conceptualized as distributed along a spectrum with various contributions of autoimmune (adaptive immune dysregulation) and autoinflammatory (innate immune dysregulation) mechanisms. Our evolving understanding of systemic immune-mediated disorders has been accompanied by an expansion in our differential diagnoses and therapeutic options. Rheumatologic disease involves both autoimmune and autoinflammatory mechanisms. Neurologic symptoms can be the first manifestation of these disorders, and familiarity with the systemic manifestations of specific diseases is essential to establish the correct diagnosis. Conversely, knowledge of the neurologic syndromes that are most likely to be associated with specific systemic disorders can help narrow the differential and increase confidence when attributing a neuropsychiatric symptom to an underlying systemic disorder.

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