Valproate-Induced Systemic Lupus Erythematosus in a Patient with Partial Trisomy of Chromosome 9 and Epilepsy |
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| Authors: | Gian Luigi Gigli† Anna Scalise‡ Flavia Pauri‡ Giulia Silvestri‡ Marina Diomedi† Fabio Placidi Maria Grazia Pomponi § Cesare Masala&#; |
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| Institution: | Clinica Neurologica, UniuersitàTor Vergata, Rome, Italy;Associazione Anni Verdi, Rome, Italy;IRCCS Cinica S. Lucia, Rome, Italy;Istituto di Genetica Medica, UniuersitàCattolica, Rome, Italy;Dipartimento Malattie Infettiue, UniuersitàLa Sapienza, Rome, Italy |
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| Abstract: | Summary: We report a mentally retarded 30–year-old woman with partial trisomy of chromosome 9 (46,XX6,+der(6)t(6,9)pat) who has had epilepsy since age 11 months. She had been treated with various combinations of drugs. After 1 year of treatment with valproate (VPA) and ethosuximide (ESM), the patient developed arthral-gias, muscle weakness, fatigue, and fever. Laboratory examination showed increased sedimentation rate, hyper-gammaglobulinemia, and high titers of antinuclear antibodies (ANA). The possibility of VPA-induced systemic lupus erythematosus (SLE) was considered. This diagnosis was supported by detection of antihistone antibodies and the HLA-DR4 antigen. VPA dosage was tapered and discontinued, with accompanying resolution of clinical, immunological and hematological signs of SLE 6 weeks after VPA discontinuation. This is the fourth reported case of VPA-induced SLE. |
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| Keywords: | Valproate Systemic lupus erythematosus Trisomy 9p Drug toxicity HLA-HR4 |
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