Key result
Lupus anticoagulant tracks with thrombotic events in familial Sneddon's syndrome while anticardiolipin antibodies remain stable.
Why the study?
The link between antiphospholipid antibodies and Sneddon's syndrome with thrombotic events remains unclear and requires sequential study in affected patients and families.
Case Report (n=13)
In a family with Sneddon's syndrome, thrombotic events were temporally related to lupus anticoagulant, and recurrences were prevented by oral anticoagulation but not aspirin.
Answers are grounded in this paper and cite their sources.
May support oral anticoagulation over aspirin for thrombosis prevention in Sneddon's syndrome with lupus anticoagulant; hypothesis-generating and requires validation.
BACKGROUND: Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions, with a hereditary transmission and unknown etiopathogenesis. A number of reports have documented a link between antiphospholipid antibodies and Sneddon's syndrome with different results. The present work was designed to sequentially study antiphospholipid antibodies in a patient with Sneddon's syndrome and her family and their potential role in thrombotic events. We used cardiolipin and a mixture of phospholipids from rabbit brain as antigen for antiphospholipid assays to determine diagnostic usefulness. CASE DESCRIPTIONS: A patient with Sneddon's syndrome and 12 available family members belonging to three generations were evaluated to determine the presence of antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) during vascular thrombotic events and asymptomatic periods. CONCLUSIONS: Our results support a temporal relation between thrombotic events in Sneddon's syndrome and lupus anticoagulant; anticardiolipin antibodies remained invariable. Our index case patient and her father could be diagnosed as having primary antiphospholipid antibody syndrome. Aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. The use of a mixture of phospholipids as antigen could present some advantages in serological studies performed in antiphospholipid syndromes.
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Lousa et al. (1994) conducted a case report in Sneddon's syndrome (n=13). Antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) was evaluated on Thrombotic events. Lupus anticoagulant showed a temporal relation with thrombotic events in a patient with Sneddon's syndrome and her family, while anticardiolipin antibodies remained invariable.
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