Systemic lupus erythematosus (SLE) is the rheumatologic disease whose neuropsychiatric manifestations have received the most attention from both clinicians and researchers. This chapter first focuses on systemic lupus erythematosus and then discusses other rheumatic disorders with the propensity for producing neuropsychiatric sequelae, including the antiphospholipid antibody syndrome (APS), Sjögren's syndrome, the vasculitides, systemic sclerosis (scleroderma), sarcoidosis, and celiac disease. The use of laboratory studies for the diagnosis of rheumatologic conditions has become increasingly refined and remains a key feature of the diagnostic process. Neuropsychological testing in these patients may facilitate the identification of specific patterns of cognitive impairment, assisting patient care and providing clues to pathophysiology. Neurologic complications in many of these diseases are rare, and our knowledge regarding diagnosis, clinical and neuroradiological presentation, and management is largely derived from case reports and case series.