Home > Journals > Panminerva Medica > Past Issues > Panminerva Medica 2017 September;59(3) > Panminerva Medica 2017 September;59(3):254-68

CURRENT ISSUE
 

JOURNAL TOOLS

eTOC
To subscribe
Submit an article
Recommend to your librarian
 

ARTICLE TOOLS

Publication history
Reprints
Permissions
Cite this article as

 

REVIEW   

Panminerva Medica 2017 September;59(3):254-68

DOI: 10.23736/S0031-0808.17.03324-9

Copyright © 2017 EDIZIONI MINERVA MEDICA

language: English

Catastrophic antiphospholipid syndrome: an update

Gerard ESPINOSA, Ignasi RODRÍGUEZ-PINTÓ, Ricard CERVERA

Department of Autoimmune Diseases, Hospital Clinic, Institut d’Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain


PDF


Catastrophic antiphospholipid syndrome (CAPS) is a rare variant that accounts for 1% of patients with APS. Despite its low frequency, the mortality-related is very high ranging from 50% of patients in the first series to 37% in the most recent data. The current knowledge of this potential devastating entity comes from the International Registry of patients with CAPS, named CAPS Registry. Small vessel thrombosis, laboratory features of microangiopathic haemolytic anemia, and development of multisystem involvement in a very short period of time are the main characteristics of this syndrome. Clinical manifestations are due to thrombosis but also, although the evidences are indirect, to excess of proinflammatory cytokines. Therefore, treatment strategy is based on the combination of anticoagulation, glucocorticoids, plasma exchange and/or intravenous immunoglobulins, the so-called triple therapy. In refractory cases or in those with initial life-threatening situation, rituximab may be an effective option. Recently, some cases of CAPS have been effectively treated with the addition of eculizumab to the triple therapy.


KEY WORDS: Antiphospholipid syndrome - Mortality - Therapeutics

top of page