Peripheral vascular occlusions leading to gangrene and amputations in antiphospholipid antibody positive patients.
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.
Asherson
Ronald A
RA
Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa. ashron@icon.co.za
Cervera
Ricard
R
Shoenfeld
Yehuda
Y
D000328:Adult; D000368:Aged; D000671:Amputation, Surgical; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000473:pathology / Q000601:surgery; D002388:Catastrophic Illness; D003240:Connective Tissue Diseases / Q000150:complications*; D005121:Extremities / Q000473:pathology / Q000601:surgery; D005260:Female; D005385:Fingers / Q000473:pathology; D005734:Gangrene / Q000209:etiology* / Q000601:surgery; D013502:General Surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D016491:Peripheral Vascular Diseases / Q000209:etiology* / Q000601:surgery; D014034:Toes / Q000473:pathology / Q000601:surgery
D017152:Antibodies, Antiphospholipid