[2016 review on catastrophic antiphospholipid syndrome].

Abstract

The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement. The vital prognosis is frequently engaged. CAPS is often precipitated by infectious diseases, surgical procedures and/or withdrawal or modification of the anticoagulation. CAPS overall mortality rate has decreased and is currently below 30%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the association of anticoagulation and steroids, plus plasma exchange and/or intravenous immunoglobulins. Cyclophosphamide is added only in patients with active systemic lupus erythematosus. The potential contribution of some additional therapies (rituximab, eculizumab or sirolimus) needs to be assessed. The prevention of CAPS is essential and is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment and the prevention with immunization of infections and the education of patients with antiphospholipid syndrome, especially for the management of oral anticoagulants.

• Authors: Nathalie Costedoat-Chalumeau, Laetitia Coutte, Véronique Le Guern, Nathalie Morel, Gaelle Leroux, Romain Paule, Luc Mouthon, Jean-Charles Piette
• Journal: Presse medicale (Paris, France : 1983) (Presse Med) Vol. 45 Issue 12 Pt 1 Pg. 1084-1092 (Dec 2016) ISSN: 2213-0276 [Electronic] France
• Vernacular Title: Syndrome catastrophique des antiphospholipides (CAPS) : revue 2016.
• PMID: 27617783 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Topics

• Antiphospholipid Syndrome (diagnosis, epidemiology, physiopathology, therapy)
• Catastrophic Illness
• Diagnosis, Differential
• Humans
• Prognosis