Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome |
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Authors: | Costallat Guilherme Lavras Appenzeller Simone Costallat Lilian Tereza Lavras |
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Institution: | 1. Medical School of Sorocaba, Pontifical Catholic University of São Paulo, Brazil;2. Faculty of Medical Sciences, State University of Campinas (UNICAMP), Campinas, Brazil;1. Spondyloarthropaties Research Group, Universidad de La Sabana – Hospital Militar Central, Bogotá, D.C., Colombia;2. CryoHoldco LATAM, Stem Medicina Regenerativa, Bogotá, D.C., Colombia;3. Universidad Javeriana, Bogotá, D.C., Colombia;4. Young Investigator – COLCIENCIAS, Colombia;1. Service de rhumatologie, université Paris-Descartes, hôpital Cochin, Paris, France;2. EA 4490, service de rhumatologie, CHU de Lille, Lille, France;3. Inserm U1059, service de rhumatologie, CHU de Saint-Étienne, Saint-Étienne, France;1. Département de rhumatologie, centre hospitalier universitaire de Saint-Étienne, Saint-Étienne, France;2. LBTO, Inserm U1059, université Jean-Monnet, Saint-Étienne, France;1. Resident of Internal Medicine, Mexican Faculty of Medicine, La Salle University, Hospital Angeles del Pedregal, Mexico City, Mexico;2. Head of the Department of Hematology, Hospital Angeles del Pedregal, Mexico City, Mexico;3. Department of Hematology, Hospital Angeles del Pedregal, Mexico City, Mexico |
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Abstract: | ObjectiveTo review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients.MethodsWe reviewed the charts of 953 SLE patients followed up regularly at our service. ES was defined as the presence of hemolytic anemia and thrombocytopenia concomitantly or sequentially. Clinical and laboratory manifestations occurring during the disease course, as well as concomitant diseases and survival was carefully reviewed.ResultsWe identified ES in 26 of 953 (2.7%) SLE patients. Twenty-three were women with mean age at SLE diagnosis of 25.7 years. Four (15%) patients had disease onset before the age of 16. In the majority of patients (92%), immune thrombocytopenia and AIHA appeared simultaneously at the beginning of SLE. Active features of SLE were a frequent finding concomitant to ES, especially arthritis (77%), malar rash (61.5%), photosensitivity (57.6%), oral ulcers (34.6%), nephritis (73%), serositis (54%), neuropsychiatric (19%) and pulmonary (15%) manifestations. In addition to this multisystemic disease, 34.6% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome. Recurrence of ES was observed in only four (15%) patients. After follow-up time of 8.72 years, 19 patients (73%) were in remission and seven (27%) patients died.DiscussionES is a rare manifestation in SLE, occurring in patients with severe multisystemic SLE manifestations. Treatment strategies frequently used in SLE contribute to longer disease remission and less frequent exacerbation than observed in the general population with ES. |
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