Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry".

Objective: To describe the clinical and laboratory features, as well as the precipitating factors, treatment and outcome of patients with catastrophic antiphospholipid syndrome (APS).

Methods: We analyzed the 280 patients included until September 2008 in the website based international registry of patients with catastrophic APS ("CAPS Registry") (http://www.med.

Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse.

The "catastrophic" variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation with a 50% mortality rate. The purpose of this paper is to review the treatment strategies and prognostic factors in patients with catastrophic APS.

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

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.

Amputation of digits or limbs in patients with antiphospholipid syndrome.

Objective: To describe the characteristics of patients with peripheral vascular disease leading to amputation of digits or limbs encountered in patients with the antiphospholipid syndrome (APS).

Methods: Twenty-one cases derived from several geographical centers (Brazil, Serbia, Italy, Israel, United Kingdom, and South Africa) are presented. The major clinical, serological, and histopathological data (where available) of this cohort are described, documented, and analyzed.

Relapsing catastrophic antiphospholipid syndrome: report of three cases.

Background: The catastrophic variant of the antiphospholipid syndrome (CAPS), also now known as Asherson's syndrome, is defined as a potential life-threatening variant of the antiphospholipid syndrome, which is characterized by multiple small-vessel thrombosis that can lead to multiorgan failure. Relapses in patients with the CAPS are very uncommon.

Objective: To describe the clinical and laboratory features of patients with relapsing episodes of CAPS.

Catastrophic antiphospholipid syndrome: lessons from the "CAPS Registry"--a tribute to the late Josep Font.

Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant, its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. In order to put together all the published case reports as well as the new diagnosed cases from all over the world, an international registry of patients with catastrophic APS ("CAPS Registry") was created in 2000 by the European Forum on Antiphospholipid Antibodies.

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.

Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases.

Background: The catastrophic variant of the antiphospholipid syndrome (APS) is a life-threatening form of presentation of this syndrome that can be triggered by several factors.

Aim: To describe the characteristics of patients who developed catastrophic APS triggered during pregnancy and puerperium.

Methods: A review of the first 255 cases collected in the website-based "CAPS Registry" was undertaken.

Intestinal involvement secondary to the antiphospholipid syndrome (APS): clinical and immunologic characteristics of 97 patients: comparison of classic and catastrophic APS.

Objective: To analyze the clinical and laboratory characteristics of 97 patients with intestinal involvement secondary to the antiphospholipid syndrome (APS) (37 patients with classic APS and 60 with catastrophic APS).

Methods: A computer-assisted (PubMed) search of the literature was performed to identify all cases of intestinal involvement associated with the APS from 1983 to December 2005. In addition, we analyzed the web-site-based international registry of patients with catastrophic APS ("CAPS Registry").

Pregnancy and puerperium are high susceptibility periods for the development of catastrophic antiphospholipid syndrome.

It is well known that antiphospholipid syndrome (APS) is associated with recurrent pregnancies losses, but is also associated with other obstetric features such as preeclampsia, uteroplacental insufficiency and preterm birth. Pregnancy is a hypercoagulable state than can be complicated by thrombosis, especially in those patients with an underlying thrombophilic disorder. Catastrophic APS is a rare form of presentation of the APS.

Lessons from the catastrophic antiphospholipid syndrome (CAPS) registry.

Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant, its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. In order to put together all the published case reports as well as the new diagnosed cases from all over the world, an international registry of patients with catastrophic APS ("CAPS Registry") was created in 2000 by the European Forum on Antiphospholipid Antibodies.

Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors.

In order to know the causes of death and the prognostic factors, our group analyzed 250 patients included until February 2005 in the web-site based international registry of patients with catastrophic antiphospholipid syndrome (APS) ("CAPS Registry") (http://www.med.ub.

Laboratory studies on pathophysiology of the catastrophic antiphospholipid syndrome.

The 'catastrophic' variant of the antiphospholipid syndrome (APS) is characterized by a diffuse thrombotic microvasculopathy. In contrast to the classical APS, single venous or arterial medium-to-large blood vessel occlusions are uncommon. The mechanisms of catastrophic APS are not clearly understood.

Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients.

Objective: To assess the main causes of death and the prognostic factors that influence mortality in patients with the catastrophic antiphospholipid syndrome (CAPS).

Methods: We analyzed the case reports of 250 patients included in the CAPS Registry up to February 2005. To identify prognostic factors for CAPS, we compared the main clinical and immunologic features and the types of treatment in the patients who died with those features in the patients who survived.

Antiphospholipid antibodies associated with malignancies: clinical and pathological characteristics of 120 patients.

Objective: To describe the different types of malignancies associated with antiphospholipid antibodies (aPL).

Methods: We performed a computer-assisted (MEDLINE, National Library of Medicine, Bethesda, MD) search of the literature from 1966 to 2003 to identify all cases of malignancies having aPL.

Results: One hundred twenty patients were found.