The correct diagnosis of central and peripheral nervous system manifestations in patients with systemic lupus erythematosus (SLE) can be challenging because of many SLE-related and non-SLE-related processes that can be present in a patient. The American College of Rheumatology (ACR) has published case definitions for 19 neuropsychiatric SLE (NPSLE) syndromes, with careful attention to important exclusion criteria. These criteria were developed for research purposes but can be helpful to clinicians with a patient who has nervous system dysfunction. This report reviews the data regarding the application of the ACR NPSLE criteria and the influence of ethnicity and disease duration on the development of NPSLE syndromes. Cognitive dysfunction and psychiatric disorders are the two most common manifestations. The work-up and treatment of nervous system syndromes are also discussed.
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