Autosplenectomy and antiphospholipid antibodies in systemic lupus erythematosus: A pathogenetic relationship? 2003

Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
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.

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.

UI MeSH Term Description Entries
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013158 Splenic Diseases Diseases involving the SPLEEN. Disease, Splenic,Diseases, Splenic,Splenic Disease
D013922 Thrombocytosis Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed) Thrombocythemia,Thrombocythemias,Thrombocytoses
D016736 Antiphospholipid Syndrome The presence of antibodies directed against phospholipids (ANTIBODIES, ANTIPHOSPHOLIPID). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (ANTIBODIES, ANTICARDIOLIPIN). Present also are high levels of lupus anticoagulant (LUPUS COAGULATION INHIBITOR). Anti-Phospholipid Antibody Syndrome,Anti-Phospholipid Syndrome,Antiphospholipid Antibody Syndrome,Hughes Syndrome,Anti Phospholipid Antibody Syndrome,Anti Phospholipid Syndrome,Antibody Syndrome, Anti-Phospholipid,Antibody Syndrome, Antiphospholipid,Antiphospholipid Antibody Syndromes,Syndrome, Anti-Phospholipid,Syndrome, Anti-Phospholipid Antibody,Syndrome, Antiphospholipid,Syndrome, Antiphospholipid Antibody,Syndrome, Hughes
D017152 Antibodies, Antiphospholipid Autoantibodies directed against phospholipids. These antibodies are characteristically found in patients with certain autoimmune diseases (e.g., SYSTEMIC LUPUS ERYTHEMATOSUS and ANTIPHOSPHOLIPID SYNDROME), some non-autoimmune diseases, and also in healthy individuals. Antiphospholipid Antibodies,Antiphospholipid Autoantibodies,Antiphospholipid Autoantibody,Phospholipid Autoantibodies,Phospholipid Autoantibody,Antiphospholipid Antibody,Antibody, Antiphospholipid,Autoantibody, Antiphospholipid,Autoantibody, Phospholipid
D017445 Skin Diseases, Vascular Skin diseases affecting or involving the cutaneous blood vessels and generally manifested as inflammation, swelling, erythema, or necrosis in the affected area. Cutaneous Vasculitis,Vascular Skin Diseases,Cutaneous Vasculitides,Skin Disease, Vascular,Vascular Skin Disease,Vasculitis, Cutaneous

Related Publications

Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
October 1996, The Journal of rheumatology,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
July 1989, The Journal of rheumatology,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
January 2000, Lupus,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
September 1994, Current opinion in rheumatology,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
January 1990, Delaware medical journal,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
May 1990, The Tokai journal of experimental and clinical medicine,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
December 1995, Pediatrics,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
December 2001, Frontiers in bioscience : a journal and virtual library,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
August 2022, International journal of molecular sciences,
Daniele Santilli, and Marcello Govoni, and Napoleone Prandini, and Nicoletta Rizzo, and Francesco Trotta
January 1992, Contributions to nephrology,
Copied contents to your clipboard!