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Occlusive vasculopathy in systemic lupus erythematosus. Association with anticardiolipin antibody
S G Greisman1, R S Thayaparan, T A Godwin
1Department of Medicine, St Luke's-Roosevelt Hospital Center, New York, NY 10019.
Insights
Systemic lupus erythematosus patients with antiphospholipid antibodies can experience catastrophic vascular occlusions. Treatment may benefit from plasmapheresis and anticoagulation, not immunosuppressants.
Area of Science:
- Rheumatology
- Immunology
- Pathology
Background:
- Systemic lupus erythematosus (SLE) and antiphospholipid antibodies (aPL) are associated with vascular events.
- Sudden occlusion of multiple blood vessels can occur in affected patients.
Observation:
- Two cases of SLE with high-titer aPL presented with acute, widespread, non-inflammatory visceral vascular occlusions.
- Histopathology differentiated these cases from typical SLE vasculitis.
Findings:
- The observed vascular occlusions were non-inflammatory.
- High-titer antiphospholipid antibodies were present in both patients.
Implications:
- Steroids and immunosuppressants may have limited efficacy due to the non-inflammatory nature of the occlusion.
- Plasmapheresis combined with anticoagulant therapy is suggested as a potential treatment strategy.
Abstract:
Patients with systemic lupus erythematosus and with antiphospholipid antibody are subject to sudden occlusion of multiple blood vessels. We describe two patients with systemic lupus erythematosus with acute, catastrophic, widespread non-inflammatory visceral vascular occlusions associated with high-titer antiphospholipid antibody. The histopathologic features clearly distinguished these patients from classic systemic lupus erythematosus vasculitis. We further suggest that, based on a probable pathogenesis related to the presence of antiphospholipid antibody, and based on the non-inflammatory vascular occlusion, steroids and other immunosuppressive medications are of limited value. Plasmapheresis along with anticoagulant therapy should be considered.
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