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Vascular disease in the antiphospholipid syndrome: a comparison with the patient population with atherosclerosis
C K Shortell1, K Ouriel, R M Green
1Department of Surgery, University of Rochester School of Medicine and Dentistry, NY.
Insights
Antiphospholipid syndrome (APS) affects young, non-smoking women, causing vascular disease and graft failure. Suspect APS in patients with unexplained arterial or venous occlusions, especially with cerebrovascular or upper extremity involvement.
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and pregnancy morbidity.
- Diagnosis relies on clinical criteria and the presence of antiphospholipid antibodies.
Purpose of the Study:
- To describe the clinical and angiographic features of arterial manifestations of APS.
- To compare demographic and clinical characteristics of APS patients with atherosclerosis patients.
Main Methods:
- Retrospective analysis of 1078 patients treated between 1987 and 1991.
- Diagnosis of APS based on clinical presentation and laboratory findings (anticardiolipin antibody, lupus anticoagulant).
- Comparison of demographic and clinical data between APS patients and a control group with atherosclerosis.
Main Results:
- APS was diagnosed in 19 of 1078 patients.
- Common manifestations included cerebral circulation involvement (stroke, TIA), upper extremity disease (Raynaud's, arterial lesions), and lower extremity disease.
- APS patients were younger, more likely to be female, non-smokers, and had higher rates of upper extremity involvement and early graft failure compared to atherosclerosis patients.
Conclusions:
- APS should be suspected in young, female, non-smokers with vascular disease, particularly those with unusual arterial or venous occlusions.
- The syndrome is associated with recurrent vascular reconstruction failure and native vessel occlusion.
- Distinctive features include cerebrovascular disease with normal extracranial carotid angiography and premature graft failure.
Abstract:
The antiphospholipid syndrome was diagnosed in 19 of 1078 patients treated between 1987 and 1991. All patients with antiphospholipid syndrome had either anticardiolipin antibody (16/19) or lupus anticoagulant (10/19); three patients had thrombocytopenia, eight patients had a prolonged partial thromboplastin time, and 10 patients had an elevated erythrocyte sedimentation rate. The most common site of involvement was the cerebral circulation (nine patients), manifested by transient ischemic attacks or stroke. Eight patients had upper extremity disease, characterized by symptoms of Raynaud's phenomenon, with angiographic lesions involving the brachial, radial, ulnar, and/or digital arteries. Lower extremity disease occurred in seven patients, with clinical presentations similar to those of atherosclerosis and varying angiographic patterns. In comparison with the population having atherosclerosis, patients with arterial manifestations of antiphospholipid syndrome were more likely to be women (13 of 19 versus 411 of 1078, p less than 0.02), were significantly younger (46.2 years versus 63.6 years, p less than 0.0001), did not smoke (1 of 19 patients versus 700 of 1078, p less than 0.0001), had a higher percentage of upper extremity involvement (8 of 18 versus 13 of 1078, p less than 0.0001), and had a higher incidence of early graft failure (9 of 12 grafts versus 13 of 371 grafts, p less than 0.0001). The syndrome is associated with the repetitive failure of vascular reconstructions and occlusion of native vessels. Antiphospholipid syndrome should therefore be suspected in young, female, nonsmokers with vascular disease, especially those with involvement of the upper extremity, cerebrovascular disease with normal findings on extracranial carotid angiography, and premature graft failure.