Amputation of digits or limbs in patients with antiphospholipid syndrome.
OBJECTIVE
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).


METHODS
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.


RESULTS
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 was detected in only 1 patient.


CONCLUSIONS
Peripheral vascular disease leading to amputation of digits or limbs is a severe complication encountered in patients with APS. In the absence of histopathology, it may be difficult to distinguish whether concomitant atherosclerotic occlusions, vasculitis, or aPL-related thrombosis of peripheral vessels is the main cause of the vascular ischemia. Treatment should, therefore, include full anticoagulation as well as corticosteroids and immunosuppression in these patients.
Asherson
Ronald A
RA
Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa. ashron@icon.co.za
Cervera
Ricard
R
Klumb
Evandro
E
Stojanovic
Ljudmila
L
Sarzi-Puttini
Piercarlo
P
Yinh
Janet
J
Bucciarelli
Silvia
S
Espinosa
Gerard
G
Levy
Roger
R
Shoenfeld
Yehuda
Y
D000328:Adult; D000368:Aged; D000671:Amputation, Surgical; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000628:therapy*; D001157:Arterial Occlusive Diseases / Q000150:complications*; D005121:Extremities / Q000098:blood supply / Q000601:surgery; D005260:Female; D005385:Fingers / Q000098:blood supply / Q000601:surgery; D006801:Humans; D007511:Ischemia / Q000601:surgery*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D008875:Middle Aged
D000925:Anticoagulants