系统性红斑狼疮中的自身脾切除与抗磷脂抗体：一种致病关系？
Autosplenectomy and antiphospholipid antibodies in systemic lupus erythematosus: A pathogenetic relationship?
作者信息
Santilli Daniele, Govoni Marcello, Prandini Napoleone, Rizzo Nicoletta, Trotta Francesco
机构信息
Unità Operativa di Reumatologia, and Servizio di Medicina Nucleare, Università degli Studi di Ferrara, Ferrara, Italy and Azienda Ospedaliera-Universitara "Arcispedale S. Anna", Ferrara, Italy.
出版信息
Semin Arthritis Rheum. 2003 Oct;33(2):125-33. doi: 10.1016/s0049-0172(03)00004-0.
OBJECTIVES
To describe a systemic lupus erythematosus (SLE) patient with functional asplenia and antiphospholipid syndrome (APS) and to review the literature to better define its pathogenesis and diagnosis, emphasizing a possible relationship with antiphospholipid antibodies (aPL).
METHODS
Descriptive report of 1 case and review of the literature by means of a MEDLINE search from 1966 to 2002.
RESULTS
A SLE patient presented with cutaneous vasculitis and an unexpected thrombocytosis which resulted from autosplenectomy. Subsequently, she developed full-blown APS. In the literature, autosplenectomy has been described only in 1 other case of APS secondary to SLE. However, clinical or laboratory features linked to aPL occurred in several other cases among the 17 cases reported with functional asplenia.
CONCLUSIONS
Autosplenectomy in SLE may be pathogenetically related to aPL. Thrombocytosis, unusual in SLE, may be a diagnostic clue of this condition. Pneumococcal vaccination is warranted to prevent life-threatening infections that frequently complicate this asplenia.
目的
描述一名患有功能性无脾症和抗磷脂综合征（APS）的系统性红斑狼疮（SLE）患者，并回顾文献以更好地明确其发病机制和诊断，重点强调与抗磷脂抗体（aPL）的可能关系。
方法
对1例病例进行描述性报告，并通过检索1966年至2002年的MEDLINE数据库对文献进行回顾。
结果
一名SLE患者出现皮肤血管炎和因自身脾切除导致的意外血小板增多症。随后，她发展为典型的APS。在文献中，仅在另一例继发于SLE的APS病例中描述过自身脾切除。然而，在报告的17例功能性无脾症病例中，其他几例出现了与aPL相关的临床或实验室特征。
结论
SLE中的自身脾切除可能在发病机制上与aPL有关。SLE中不常见的血小板增多症可能是这种情况的诊断线索。有必要进行肺炎球菌疫苗接种以预防常使这种无脾症复杂化的危及生命的感染。
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