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Abstract

The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus.

Ulas D Bayraktar, Doruk Erkan, Silvia Bucciarelli, Gerard Espinosa, Ronald Asherson and Catastrophic Antiphospholipid Syndrome Project Group
The Journal of Rheumatology February 2007, 34 (2) 346-352;
Ulas D Bayraktar
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Doruk Erkan
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Silvia Bucciarelli
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Gerard Espinosa
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Ronald Asherson
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Abstract

OBJECTIVE: To compare the clinical spectrum of patients with primary catastrophic antiphospholipid syndrome (P-CAPS) to those with systemic lupus erythematosus-associated CAPS (SLE-CAPS). METHODS: We used the Internet-based CAPS Registry to compare the demographic, clinical, and laboratory characteristics of 127 P-CAPS patients to 103 SLE-CAPS patients. In a logistic regression analysis, we also determined the poor prognostic factors for mortality. RESULTS: At the time of CAPS diagnosis, compared to patients with P-CAPS, those with SLE-CAPS were more likely to be female and younger; have cerebral and pancreatic involvement; receive corticosteroids and cyclophosphamide; demonstrate a lower prevalence of high titer (> or = 80 U) IgG anticardiolipin antibody; and have a higher risk for mortality after adjusting for age, sex, organ involvement, and treatment. Based on a logistic regression analysis, cyclophosphamide use was associated with increased mortality in P-CAPS but improved survival in SLE-CAPS patients. CONCLUSION: SLE is a poor prognostic factor in patients with CAPS and cyclophosphamide may be beneficial in those with SLE-CAPS.

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The Journal of Rheumatology
Vol. 34, Issue 2
1 Feb 2007
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The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus.
Ulas D Bayraktar, Doruk Erkan, Silvia Bucciarelli, Gerard Espinosa, Ronald Asherson, Catastrophic Antiphospholipid Syndrome Project Group
The Journal of Rheumatology Feb 2007, 34 (2) 346-352;

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The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus.
Ulas D Bayraktar, Doruk Erkan, Silvia Bucciarelli, Gerard Espinosa, Ronald Asherson, Catastrophic Antiphospholipid Syndrome Project Group
The Journal of Rheumatology Feb 2007, 34 (2) 346-352;
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