Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors

Seminars in Thrombosis and Hemostasis
G EspinosaR Cervera

Abstract

The catastrophic variant of the antiphospholipid syndrome (APS) is a condition characterized by multiple vascular occlusive events, usually affecting small vessels and evolving over a short period of time, together with laboratory confirmation of the presence of antiphospholipid antibodies. The pathogenesis of catastrophic APS is not completely understood. The mortality rate was ~50% in the earliest published series, but recently it has clearly fallen by some 20% due to the use, as first-line therapies, of full anticoagulation, corticosteroids, plasma exchanges, and intravenous immunoglobulins. Cerebral involvement has been identified as the main cause of death, being present in one third of patients, and consisting mainly of stroke, cerebral hemorrhage and encephalopathy, followed by cardiac involvement and infection. The only identified prognostic factor for a higher mortality rate is the presence of systemic lupus erythematosus.

Citations

Dec 18, 2008·Clinical Reviews in Allergy & Immunology·Mario García-CarrascoRicard Cervera
Feb 25, 2010·Rheumatology·Prashantha M Vaitla, Elizabeth M McDermott
Feb 1, 2011·Clinical Chemistry and Laboratory Medicine : CCLM·Emmanuel J Favaloro, Richard C W Wong
Dec 14, 2011·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Gloria von GeldernKyra Becker
Oct 16, 2012·Journal of the American Academy of Dermatology·Leilani R TownsendStuart R Gildenberg
May 17, 2011·Autoimmunity Reviews·Gerard EspinosaRicard Cervera
Dec 9, 2008·Pediatric Blood & Cancer·Amulya A Nageswara RaoVilmarie Rodriguez
Mar 18, 2011·Dermatologic Therapy·Ashley Wysong, Priya Venkatesan
Aug 30, 2012·Journal of Medical Virology·M GoeijenbierE C M van Gorp
Jun 23, 2010·Journal of Clinical Apheresis·Zbigniew M SzczepiorkowskiUNKNOWN Apheresis Applications Committee of the American Society for Apheresis
Dec 20, 2014·Proteomics. Clinical Applications·Priscila Camillo TeixeiraPaul Cutler
May 15, 2012·The Journal of Pediatrics·Marilyn J Manco-JohnsonPeter M Mourani
Jan 22, 2015·Current Rheumatology Reports·Ignasi Rodríguez-PintóRicard Cervera
May 19, 2010·Expert Review of Clinical Immunology·Jozélio F de CarvalhoYehuda Shoenfeld
May 14, 2009·Current Opinion in Pulmonary Medicine·Gerard Espinosa, Ricard Cervera
Apr 14, 2010·Nature Reviews. Rheumatology·Gerard Espinosa, Ricard Cervera
May 29, 2018·Case Reports in Obstetrics and Gynecology·Eryk Hakman, Sasha Mikhael
May 16, 2019·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Brian NguyenJason A Ferreira
May 1, 2010·Auto- Immunity Highlights·Emmanuel J Favaloro, Richard C W Wong
Jun 19, 2012·The Korean Journal of Internal Medicine·Hayne Cho ParkSuhnggwon Kim
Jul 29, 2009·Current Opinion in Pulmonary Medicine

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Polyendocrinopathies

Autoimmune polyendocrinopathies, also called polyglandular autoimmune syndromes (PGASs), or polyendocrine autoimmune syndromes(PASs), are a heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected. Discover the latest research on autoimmune polyendocrinopathies here.

Acute Stroke

A stroke occurs when blood supply to the brain is interrupted depriving the brain of oxygen and nutrients. This feed focuses cerebrovascular accidents including ischemic and paralytic stroke.

Autoimmune Polyendocrine Syndromes

This feed focuses on a rare genetic condition called Autoimmune Polyendocrine Syndromes, which are characterized by autoantibodies against multiple endocrine organs. This can lead to Type I Diabetes.

Antiphospholipid Syndrome

Antiphospholipid syndrome or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by the presence of antibodies directed against phospholipids.