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2016 ; 28
(3
): 218-27
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Treatment of catastrophic antiphospholipid syndrome
#MMPMID26927441
Kazzaz NM
; McCune WJ
; Knight JS
Curr Opin Rheumatol
2016[May]; 28
(3
): 218-27
PMID26927441
show ga
PURPOSE OF REVIEW: Catastrophic antiphospholipid syndrome (CAPS) is a severe
manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of
patients with APS, the condition is frequently fatal if not recognized and
treated early. Here, we will review the current approach to diagnosis and
treatment of CAPS. RECENT FINDINGS: Data from the international 'CAPS registry',
spearheaded by the European Forum on Antiphospholipid Antibodies, have improved
our understanding of at-risk patients, typical clinical features, and
precipitating diagnoses. Current guidelines also continue to support the role of
anticoagulants and glucocorticoids as foundation therapy in all patients.
Finally, new basic science and case series suggest that novel therapies, such as
rituximab and eculizumab, warrant further study. SUMMARY: Attention to associated
diagnoses, such as infection and systemic lupus erythematosus (SLE), is critical
at the time of diagnosis. All patients should be treated with anticoagulants,
corticosteroids, and possibly plasma exchange. In patients with SLE,
cyclophosphamide should be considered. In refractory or relapsing cases, new
therapies, such as rituximab and possibly eculizumab, may be options, but need
further study.
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