Evans syndrome and Systemic Lupus Erythematosus: Clinical presentation and outcome

Autor(es)

Costallat, Guilherme Lavras ^[1] ; Appenzeller, Simone ^[2] ; Lavras Costallat, Lilian Tereza ^[2]

Afiliação do(s) autor(es)

^[1] Pontifical Catholic Univ Sao Paulo, Med Sch Sorocaba, Sao Paulo - Brazil

^[2] State Univ Campinas UNICAMP, Fac Med Sci, Campinas, SP - Brazil

• Tipo de documento: Artigo Científico
• Fonte: JOINT BONE SPINE; v. 79, n. 4, p. 362-364, JUL 2012.
• Citações Web of Science: 10

Resumo

Objective: To review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients. Methods: We 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. Results: We 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. Discussion: ES 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. (C) 2011 Societe francaise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. (AU)

• Processo FAPESP: 09/11076-2 - Níveis de interferon alfa, gama e TNF alfa em pacientes lúpicos: associações com manifestações clínicas
• Beneficiário: Mariana Postal Zink de Souza
• Modalidade de apoio: Bolsas no Brasil - Mestrado