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

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.