J Saison1, N Costedoat-Chalumeau2, D Maucort-Boulch3, J Iwaz3, R Marignier4, P Cacoub5, D Vital-Durand6, A Hot7, J Tebib8, O Aumaitre9, N Schleinitz10, F Sarrot-Reynauld11, C Broussolle1, P Sève12. 1. Département de Médecine Interne, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France Université de Lyon, Université Lyon 1, Lyon, France. 2. Centre maladies rares, Service de médecine interne, Hôpital Cochin, Paris, France Université Paris Descartes, Paris, France. 3. Université de Lyon, Université Lyon 1, Lyon, France Service de Biostatistique, Hospices Civils de Lyon, Lyon, France CNRS UMR 5558, Equipe Biostatistique Santé, Pierre-Bénite, France. 4. Département de Neurologie, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, Bron, France. 5. i3 Immunologie-Immunopathologie-Immunotherapie, (UMR 7211-Université Pierre et Marie Curie/CNRS, INSERM U 959), Paris, France. 6. Département de Médecine Interne, Hôpital Lyon Sud, Hospices Civils de Lyon, Lyon, France. 7. Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France. 8. Département de Rhumatologie, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France. 9. Département de Médecine Interne, CHU Gabriel-Montpied, Clermont-Ferrand, France. 10. Département de Médecine Interne, Hôpital de la Conception, Assistance Publique-Hôpitaux de Marseille, Marseille, France. 11. Département de Médecine Interne, Hôpital Albert Michalon, Grenoble, France. 12. Département de Médecine Interne, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France Université de Lyon, Université Lyon 1, Lyon, France pascal.seve@chu-lyon.fr.
Abstract
BACKGROUND: Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE). This retrospective multicentre study identifies the prognostic factors in a relatively large patient series. PATIENTS AND METHODS: Twenty patients fulfilled the SLE criteria of the ACR classification and the Transverse Myelitis Consortium Working Group. A severe neurological flare was defined as muscle strength grade <3/5 in more than half the muscle groups at the motor neurological level. Inability to run or another significant ambulation-unrelated disability was considered as 'unfavourable neurological outcome'. RESULTS: Myelitis was the first SLE symptom in 12 patients; in the eight others, it occurred 8.6 years (median delay) after SLE onset. Eleven patients presented severe neurological impairments. The treatment included corticosteroids in all patients associated with intravenous cyclophosphamide in 11 and/or hydroxychloroquine in 14. Unfavourable outcomes were observed in 53% of the patients at six months and in 28% at end of follow-up (median: 5.9 years). An initial severe neurological impairment and no cyclophosphamide use were associated with unfavourable neurological outcomes at six months and at end of follow-up, respectively. CONCLUSION: Transverse myelitis may reveal SLE or occur more than 10 years after SLE diagnosis. The initial severity of the neurological flare (with paraplegia) is the main prognostic marker. The study provides arguments for cyclophosphamide use.
BACKGROUND:Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE). This retrospective multicentre study identifies the prognostic factors in a relatively large patient series. PATIENTS AND METHODS: Twenty patients fulfilled the SLE criteria of the ACR classification and the Transverse Myelitis Consortium Working Group. A severe neurological flare was defined as muscle strength grade <3/5 in more than half the muscle groups at the motor neurological level. Inability to run or another significant ambulation-unrelated disability was considered as 'unfavourable neurological outcome'. RESULTS:Myelitis was the first SLE symptom in 12 patients; in the eight others, it occurred 8.6 years (median delay) after SLE onset. Eleven patients presented severe neurological impairments. The treatment included corticosteroids in all patients associated with intravenous cyclophosphamide in 11 and/or hydroxychloroquine in 14. Unfavourable outcomes were observed in 53% of the patients at six months and in 28% at end of follow-up (median: 5.9 years). An initial severe neurological impairment and no cyclophosphamide use were associated with unfavourable neurological outcomes at six months and at end of follow-up, respectively. CONCLUSION:Transverse myelitis may reveal SLE or occur more than 10 years after SLE diagnosis. The initial severity of the neurological flare (with paraplegia) is the main prognostic marker. The study provides arguments for cyclophosphamide use.
Authors: Javier Pablo Hryb; Edson Chiganer; Edgar Carnero Contentti; José Luis Di Pace; Carmen Lessa; Mónica Beatriz Perassolo Journal: Spinal Cord Ser Cases Date: 2016-07-07