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Autosplenectomy and antiphospholipid antibodies in systemic lupus erythematosus: A pathogenetic relationship?
Daniele Santilli1, Marcello Govoni, Napoleone Prandini
1Unità Operativa di Reumatologia, and Servizio di Medicina Nucleare, Università degli Studi di Ferrara, Ferrara, Italy and Azienda Ospedaliera-Universitara "Arcispedale S. Anna", Ferrara, Italy.
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.
