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Neuropsychiatric Manifestations in Pediatric Systemic Lupus Erythematosus and Association with Antiphospholipid Antibodies
LIORA HAREL, CHRISTY SANDBORG, TZIELAN LEE, and EMILY von SCHEVEN ABSTRACT. Objective. To determine the prevalence of neuropsychiatric (NP) manifestations in children with systemic lupus erythematosus (SLE) using the 1999 American College of Rheumatology case definitions for NP syndromes in SLE, and their association with antiphospholipid antibodies (aPL). Methods. We performed a retrospective cohort study of 106 pediatric and adolescent SLE patients at 2 academic medical centers. Clinical and laboratory data were obtained by medical record review. All aPL testing was performed in standard clinical laboratories. Results. Twenty-five patients (23.6%) had NP manifestations, including seizures (9.4%), headaches (4.7%), mood disorders (4.7%), cognitive dysfunction (4.7%), cerebrovascular accident (CVA), psychosis and pseudotumor (2.8% each), aseptic meningitis (0.9%), acute confusional state (0.9%), anxiety (0.9%), and cranial neuropathy (0.9%). NP events were not necessarily accompanied by an SLE flare. aPL were positive in 70% of all SLE patients, including anticardiolipin antibodies (aCL) in 64%, aCL IgG in 56%, aCL IgM in 35%, rapid plasma reagin or Venereal Disease Research Laboratory test in 13%, and lupus anticoagulant (LAC) in 18%. The only significant association between NP manifestations and aPL was for CVA and IgM aCL (p=0.03). LAC was slightly more common among patients with NP events, and the finding of LAC on more than one occasion was significantly associated with developing a NP event (p = 0.01). Conclusion. NP manifestations occur in about one-fourth of children with SLE, are an early event in the course of the disease, and are not necessarily accompanied by an SLE flare. Seizures are the most frequent symptom. Although aPL are common, their association with NP events, unlike in adults, is weak, except for CVA, suggesting a different pathogenic mechanism for NP manifestations in pediatric SLE. (First Release July 15 2006; J Rheumatol 2006;33:1873–7 ) Key Indexing Terms:
SYSTEMIC LUPUS ERYTHEMATOSUS
From the Department of Pediatrics C, Schneider Children's Medical Center of Israel, Petach Tikvah, and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Pediatrics, Division of Rheumatology, Lucille Packard Children's Medical Center, Stanford University, Stanford, California; and Pediatric Immunology/ Rheumatology, Children's Medical Center, University of California, San Francisco, California, USA. L. Harel, MD, Department of Pediatrics C, Schneider Children's Medical Center of Israel and Sackler School of Medicine, Tel Aviv University; C. Sandborg, MD; T.C. Lee, MD, Department of Pediatrics, Division of Rheumatology, Lucille Packard Children's Medical Center, Stanford University; E. von Scheven, MD, Pediatric Immunology/Rheumatology, Children's Medical Center, University of California. Address reprint requests to Dr. L. Harel, Department of Pediatrics C, Schneider Children's Medical Center of Israel, Petah Tiqwa 49202, Israel. E-mail: liorahar@clalit.org.il Accepted for publication April 7, 2006.
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