Autosplenectomy and antiphospholipid antibodies in systemic lupus erythematosus: A pathogenetic relationship?
OBJECTIVE
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.
Santilli
Daniele
D
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.
Govoni
Marcello
M
Prandini
Napoleone
N
Rizzo
Nicoletta
N
Trotta
Francesco
F
D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D017445:Skin Diseases, Vascular / Q000276:immunology; D013158:Splenic Diseases / Q000276:immunology*; D013922:Thrombocytosis / Q000276:immunology
D017152:Antibodies, Antiphospholipid