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.


CONCLUSIONS
SLE is a poor prognostic factor in patients with CAPS and cyclophosphamide may be beneficial in those with SLE-CAPS.
Bayraktar
Ulas D
UD
Interfaith Medical Center, Brooklyn, New York, USA.
Erkan
Doruk
D
Bucciarelli
Silvia
S
Espinosa
Gerard
G
Asherson
Ronald
R
D000328:Adult; D016736:Antiphospholipid Syndrome / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology*; D002388:Catastrophic Illness / Q000401:mortality; D015897:Comorbidity; D005260:Female; D014943:Global Health; D006801:Humans; D007391:International Cooperation; D020407:Internet; D016015:Logistic Models; D008180:Lupus Erythematosus, Systemic / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology*; D008297:Male; D011379:Prognosis; D012042:Registries; D015996:Survival Rate