What is the epidemiology of CNS lupus?

Updated: May 04, 2021
  • Author: Pradeep C Bollu, MD; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM  more...
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Answer

According to a set of definitions of 19 neuropsychiatric systemic lupus erythematosus (NPSLE) syndromes and their diagnostic criteria from the American College of Rheumatology (ACR), less than 40-50% of events are due to underlying CNS lupus activity (primary NPSLE). The rest are indirectly associated to the disease and can be the consequence of metabolic disturbances, infections, or drug effects (secondary NPSLE). [4, 5, 6]

Data from large cohorts suggest prevalence rates of approximately 30–40% for NPSLE. [7, 8] Further studies show NPSLE is at least as common in children as it is in adults. [9, 10]

A three-year prospective study of 370 SLE patients with no previous history of CNS involvement determined that clinically severe CNS involvement is rare in SLE patients, accounting for 7.8/100 person years. [11]


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