Abstract
To describe the characteristics of patients with peripheral vascular disease leading to amputation of digits or limbs encountered in patients with the antiphospholipid syndrome (APS). Twenty-one cases derived from several geographical centers (Brazil, Serbia, Italy, Israel, United Kingdom, and South Africa) are presented. The major clinical, serological, and histopathological data (where available) of this cohort are described, documented, and analyzed. Patients were suffering mainly from systemic lupus erythematosus (9 patients) or primary APS (8 patients). Peripheral vascular occlusions occurred during the course of the catastrophic APS in 5 patients. The vascular occlusions occurred both early and very late in the course of the disease (time after APS diagnosis, 0-38 years). Vasculitis was present in 7 patients and 5 demonstrated the typical antiphospholipid antibody (aPL)--vasculopathy with complicating bland thrombosis. Myocardial infarctions had occurred in 4 patients but it was not possible to determine whether they suffered from premature atherosclerotic disease or whether the infarctions were aPL-related. The appearance of livedo reticularis preceding the arterial thrombosis was noted in 9 patients. Cryoglobulinemia wa...Continue Reading
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