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• W2073041748 abstract "SNEDDON SYNDROME (SNS) is a hypercoagulable state associated with ischemic cerebrovascular events and livedo reticularis. 1 Sneddon I.B. Cerebrovascular lesions and livedo reticularis. Br J Dermatol. 1965; 77: 180-185 Crossref PubMed Scopus (341) Google Scholar Other organ manifestations are heart valve disease and renal insufficiency. 2 Frances C. Piette J.C. The mystery of Sneddon syndrome Relationship with antiphospholipid syndrome and systemic lupus erythematosus. J Autoimmun. 2000; 15: 139-143 Crossref PubMed Scopus (89) Google Scholar Although the underlying mechanism is poorly understood, the presence of antiphospholipid (APL) or anticardiolipin (ACL) antibodies may cause thrombotic events and valvulopathy. 3 Frances C. Papo T. Wechsler B. et al. Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore). 1999; 78: 209-219 Crossref PubMed Scopus (179) Google Scholar Irregular thickening of the mitral and/or aortic valve leads to valvular dysfunction and eventually stenosis, or most frequently, insufficiency. Systemic hypertension is present in approximately half of the patients, and venous thrombosis may occur in 15% of the patients with SNS. 3 Frances C. Papo T. Wechsler B. et al. Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore). 1999; 78: 209-219 Crossref PubMed Scopus (179) Google Scholar Cortical and subcortical cerebral abnormalities suggest arterial ischemic events. 2 Frances C. Piette J.C. The mystery of Sneddon syndrome Relationship with antiphospholipid syndrome and systemic lupus erythematosus. J Autoimmun. 2000; 15: 139-143 Crossref PubMed Scopus (89) Google Scholar , 3 Frances C. Papo T. Wechsler B. et al. Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore). 1999; 78: 209-219 Crossref PubMed Scopus (179) Google Scholar Diffuse corticosubcortical atrophy is the late-term finding of the disease. In addition, progressive vascular dementia has been reported. 3 Frances C. Papo T. Wechsler B. et al. Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore). 1999; 78: 209-219 Crossref PubMed Scopus (179) Google Scholar The incidence of positive APL antibodies ranges from 0% to 85% in various series among the patients having the disease. 4 Zelger B. Sepp N. Stockhammer G. et al. Sneddon’s syndrome A long-term follow-up of 21 patients. Arch Dermatol. 1993; 129: 437-447 Crossref PubMed Scopus (127) Google Scholar , 5 Kalashnikova L.A. Nasonov E.L. Borisenko V.V. et al. Sneddon’s syndrome Cardiac pathology and antiphospholipid antibodies. Clin Exp Rheumatol. 1991; 9: 357-361 PubMed Google Scholar SNS patients with high ACL antibody titers are defined as ACL positive. The prevalence of SNS is unknown; however, the incidence of APL syndrome in the general population is reported to be 2%. 6 Shapiro S.S. Thiagaran P. Lupus anticoagulants. Prog Hemost Throm. 1982; 6: 263-285 PubMed Google Scholar Optimal management of these patients is still in debate. 3 Frances C. Papo T. Wechsler B. et al. Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore). 1999; 78: 209-219 Crossref PubMed Scopus (179) Google Scholar" @default.
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• W2073041748 date "2005-10-01" @default.
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• W2073041748 title "Mitral Valve Replacement in a Patient With Sneddon Syndrome" @default.
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