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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, and RONALD ASHERSON, for the Catastrophic Antiphospholipid Syndrome Project Group 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 (≥ 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. (J Rheumatol 2007;34:346-52) Key Indexing Terms:
CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME From Interfaith Medical Center, Brooklyn, New York; The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, New York, USA; Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain; and Division of Immunology, School of Pathology, University of Witwatersrand, Johannesburg, South Africa. U.D. Bayraktar, MD, Internal Medicine Resident, Interfaith Medical Center; D. Erkan, MD, Assistant Professor of Medicine, The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery; S. Bucciarelli, MD, Research Fellow; G. Espinosa, MD, PhD, Rheumatology Specialist, Department of Autoimmune Diseases, Hospital Clinic, Barcelona; R. Asherson, MD, Professor of Immunology, Division of Immunology, School of Pathology, University of Witwatersrand. Address reprint requests to Dr. D. Erkan, The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021. E-mail: derkan@pol.net Accepted for publication September 29, 2006.
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