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The Incidence and Prevalence of Neuropsychiatric Syndromes in Pediatric Onset Systemic Lupus Erythematosus
WILMER L. SIBBITT Jr, JOHN R. BRANDT, COURTNEY R. JOHNSON, MARCOS E. MALDONADO, SAMIR R. PATEL, COREY C. FORD, ARTHUR D. BANKHURST, and WILLIAM M. BROOKS
ABSTRACT. Methods. Seventy-five pediatric onset patients with SLE including Native American, Asian, Black, Spanish-American, and Caucasian subjects were evaluated prospectively and cross sectionally. During the 6 year study, 55 patients became inpatients. Subjects underwent medical interview, physical examination, laboratory review, neuropsychiatric inventory, and chart review. Classification of NPSLE was accomplished with the 1999 ACR NPSLE case definitions. Results. Prospectively, NPSLE occurred in 95% of pediatric SLE patients and was more common than glomerulonephritis (55%; p £ 0.0001). NPSLE prevalence (%) and incidence (event/person/yr) were as follows: headache 72%, 95; mood disorder 57%, 0.41; cognitive disorder 55%, 0.49; seizure disorder 51%, 0.94; acute confusional state 35%, 0.6; anxiety disorder 21%, 0.28; peripheral nervous system disorder 15%, 0.16; cerebrovascular disease 12%, 0.32; psychosis 12%, 0.16; chorea 7%, 0.01; demyelinating syndrome 4%, 0.01; and myelopathy 1%, 0.001. Cross sectionally, active NPSLE was present in 93% of inpatients and 69% of outpatients. When only serious forms of NPSLE were considered (stroke, seizures, major cognitive disorder, chorea, psychosis, major depression, acute confusional state), serious or life-threatening NPSLE occurred in 76% of all SLE subjects prospectively, and in 85% and 40% of inpatients and outpatients cross sectionally, which in each instance was more common than glomerulonephritis (p £ 0.001). Conclusion. NPSLE is one of the most common serious complications of pediatric SLE, and is particularly increased in pediatric inpatients. (J Rheumatol 2002;29:1536-42) Key Indexing Terms:
SYSTEMIC LUPUS ERYTHEMATOSUS From the Clinical and Magnetic Resonance Research Center, Departments of Internal Medicine, Pediatrics, Neurology, and Neurosciences, The University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. Supported by research grant RO1 NS 35708 from the National Institutes of Health. W.L. Sibbitt Jr, MD, Professor of Internal Medicine and Neurology, Division of Rheumatology, Departments of Internal Medicine and Neurology; J.R. Brandt, MD, Associate Professor of Pediatrics; C.R. Johnson, MD, Associate Professor of Pediatrics, Department of Pediatrics; M.E. Maldonado, MD, Rheumatology Fellow; S.R. Patel, MD, Rheumatology Fellow, Department of Internal Medicine; C.C. Ford, MD, PhD, Professor of Neurology, Department of Neurology; A.D. Bankhurst, MD, Professor of Rheumatology, Department of Internal Medicine; W.M. Brooks, PhD, Research Professor, Department of Neurosciences. Address reprint requests to Dr. W.L. Sibbitt Jr, Department of Internal Medicine, 5th Floor ACC, University of New Mexico Health Sciences Center, Albuquerque, NM 87131, USA. Submitted April 10, 2001; revision accepted January 17, 2002. |