PT - JOURNAL ARTICLE AU - Ulas D Bayraktar AU - Doruk Erkan AU - Silvia Bucciarelli AU - Gerard Espinosa AU - Ronald Asherson AU - Catastrophic Antiphospholipid Syndrome Project Group TI - The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. DP - 2007 Feb 01 TA - The Journal of Rheumatology PG - 346--352 VI - 34 IP - 2 4099 - http://www.jrheum.org/content/34/2/346.short 4100 - http://www.jrheum.org/content/34/2/346.full SO - J Rheumatol2007 Feb 01; 34 AB - 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.