Vasculitis and vasculopathy
Amputation of Digits or Limbs in Patients with Antiphospholipid Syndrome

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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.

Section snippets

Materials and Methods

Twenty-one cases with gangrene of digits or limbs accompanied by elevations of aPL and requiring amputations were collected from several centers of different countries, including Brazil, Serbia, Italy, South Africa, United Kingdom, United States, and Israel. Important features of their clinical histories and pertinent investigations were documented. Immunological testing was performed by local laboratories according to accepted international methodologies. Five representative case histories are

Case 4

In August 1989, a 25-year-old white Brazilian woman presented with polyarthralgias, lymphadenopathy, splenomegaly, low-grade fever, livedo reticularis and Raynaud's phenomenon. She had no personal or familiar history of vaso-occlusive disease and/or dyslipidemia. She had never been pregnant, used estrogens, or smoked. The left posterior tibial pulse was not palpable and a small digital occlusion of the left toe was present. She had Coombs-positive hemolytic anemia, lymphopenia, positive

Results

The clinical and serological features of our 21 patients are summarized in Table 1, Table 2; cases are grouped according to their origin. Other features of the APS are noted, as well as histopathology when available. Some histories are more detailed than others, mainly because of the availability of data.

The initial diagnoses in the 21 cases were of SLE (9 patients), primary APS (8 patients), “lupus-like” disease (3 patients), and discoid lupus (1 patient) (Table 1). In 5 patients, “definite”

Discussion

The first communication stressing that arterial occlusions (eg, strokes) might be linked to the APS appeared in 1983 (3). Involvement of other noncerebral arteries (both large and small) was subsequently documented. Small-vessel occlusions (eg, glomerular, retinal, nailfold) are included in the latest consensus statement of APS (4). When these small-vessel occlusions dominate the clinical picture and affect vessels supplying major organs, in particular, the term catastrophic APS or Asherson's

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