Peripheral Vascular Occlusions Leading to Gangrene and Amputations in Antiphospholipid Antibody Positive Patients

Abstract

Twenty-one cases from several medical centers (Brazil, Italy, Serbia, South Africa, Israel, and the United Kingdom) with severe peripheral vascular disease progressing to amputations of limbs/digits, all of whom tested positive for antiphospholipid antibodies, are documented. The patients were suffering from either systemic lupus erythematosus, discoid LE, "primary" antiphospholipid syndrome (PAPS), "lupus-like" disease, undifferentiated connective tissue disease. A high frequency of livedo reticularis preceding the arterial occlusions in our series of patients who subsequently progressed to ischemic necrosis and amputation of limbs/digits was noted. Five of the 10 patients, in whom histopathological studies had been performed, demonstrated the typical vasculopathy seen with the antiphospholipid syndrome ("APS vasculopathy"). Complicating vasculitis was present in seven of the patients. Five of the patients developed severe peripheral vascular disease during the course of the catastrophic antiphospholipid (Asherson's) syndrome.

Digital gangrene as the initial presentation of systemic lupus erythematosus

“…The former found a cumulative incidence of digital gangrene in SLE to be 1.3%. Asherson et al 8 had reported only 27 cases of this association as of 1996. Eighteen cases of lupus digital gangrene had also been reported among the Chinese population as of 2009,9 while 20 cases were reported among a cohort of 344 Indian SLE patients 10.…”

The physiopathology of the catastrophic antiphospholipid (Asherson's) syndrome: Compelling evidence

“…cell adhesion molecule-1 (VCAM-1) [38]. An additional study reported a high frequency of livedo reticularis preceding the arterial occlusions and the subsequent development of ischemic necrosis and amputation of limbs/digits (cAPS), in patients positive for antiphospholipid antibodies [39]. In addition a complex gene expression of multiple proinflammatory cytokines, and prothrombotic proteins in response to the binding of anti-b2-GPI antibodies to ECs has been reported in studies in vitro [40].…”

Antiphospholipid Syndrome

“…8 Small-vessel occlusions including those in the nail fold are not infrequent, and Raynaud's phenomenon and livedo reticularis might appear, together or separately, preceding digital or limb ischemia. 8 Splinter hemorrhages can also be seen but are rare (0.7% of patients in the Euro-Phospholipid project).…”

“…8 Small-vessel occlusions including those in the nail fold are not infrequent, and Raynaud's phenomenon and livedo reticularis might appear, together or separately, preceding digital or limb ischemia. 8 Splinter hemorrhages can also be seen but are rare (0.7% of patients in the Euro-Phospholipid project). 1,3 During a prospective observational study of 1,000 consecutive APS patients, Cervera et al 2 found stroke (2.4%), transient ischemic attack (2.3%), deep venous thromboses (2.1%), and pulmonary emboli (2.1%) to be the most common manifestations of vascular occlusion during the 5-year course.…”

“…The concern for an inflammatory component should arise in the presence of concomitant cutaneous vascular disorders. 8 Other risk factors for atherosclerosis or hypercoagulability should be aggressively managed to reduce patient risk of further vascular occlusions.…”