The physiopathology of the catastrophic antiphospholipid (Asherson's) syndrome: compelling evidence.

Catastrophic antiphospholipid (Asherson's) syndrome (cAPS) was described in the past as a severe variant of the antiphospholipid syndrome (APS). Currently growing evidence suggests it is a unique condition. This statement is based on several clinical and physiopathological features that although not well understood define cAPS by itself.

Catastrophic antiphospholipid (Asherson's) syndrome.

Catastrophic antiphospholipid syndrome is characterized by multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions, and laboratory confirmation of the presence of antiphospholipid antibodies. Knowledge of its treatment is vital as the outcome can be lethal.

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.

Antiphospholipid antibodies and the antiphospholipid syndrome: clinical significance and treatment.

This article provides a review of the various types of antiphospholipid (aPL) antibodies and antiphospholipid syndromes, their prevalence, presumed origin, relationship to autoimmunity in general, and their role in the body's defenses and apoptosis. New hypotheses such as the role of antibodies to beta2 glycoprotein I (beta2GPI) and the signaling of toll-like receptors are also discussed, as is the spectrum of clinical manifestations associated with the demonstration of these antibodies, now assumed to be "pathogenic." A distinction is made between antibodies present in sera of patients with a variety of microangiopathic syndromes (MAPS; e.

The primary, secondary, catastrophic, and seronegative variants of the antiphospholipid syndrome: a personal history long in the making.

Although many of the clinical features accompanying lupus anticoagulant positivity were documented in the early 1960s and many "non-lupus patients" were also published, it was not until the discovery of antibodies to cardiolipin in the 1980s that the existence and true ramifications of a distinct antiphospholipid syndrome was defined. A primary syndrome was in fact recognized in 1985 by the author while at the Hammersmith Hospital and comprised 25 patients who conformed to this new subset of disease, which has now overtaken lupus-associated (secondary) antiphospholipid syndromes in frequency. However, publication of this important milestone was in fact prevented, because of the purveying dogma at that time that "these patients were all suffering from 'lupus,'" which history has since proved to be incorrect.

A case of adult-onset Satoyoshi syndrome with gastric ulceration and eosinophilic enteritis.

Background: The patient was misdiagnosed as having Sjögren's syndrome (on the basis of a lower-limb rash and dry eyes and mouth) in 1999, and then as having systemic lupus erythematosus (on the basis of hair loss and a high antinuclear antibody titer) in 2005. Total alopecia, muscular spasms and diarrhea developed over the following 2 years, and the patient experienced gastric ulceration in 2006. A rheumatologic opinion was sought in 2007.

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.

Microvascular and microangiopathic antiphospholipid-associated syndromes ("MAPS"): semantic or antisemantic?

Small vessel occlusions may occur as part of the vascular manifestations of the Antiphospholipid Syndrome (APS) and may affect glomerular, skin, retinal, bowel, hepatic or pulmonary vessels. These thrombotic lesions are proven (usually by biopsy, surgical procedures, at autopsy or by specialized techniques e.g.

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.

Pulmonary hypertension, antiphospholipid antibodies, and syndromes.

Antiphospholipid antibodies have been associated with two types of pulmonary hypertension (PHT), the thromboembolic type, after deep venous thromboses in the lower limbs complicated by pulmonary embolism and the "primary" plexogenic type. The PHT may occur in the absence of any other manifestations of the antiphospholipid syndrome (APS), and cases have been recorded with very high levels of antiphospholipid antibodies. It may also accompany systemic lupus erythematosus (SLE) and may manifest with or without other features of the APS.

Autoantibodies in nonautoimmune individuals during infections.

Infections can act as environmental triggers inducing or promoting autoimmune disease in genetically predisposed individuals. Identification of microbial peptides similar to self-tissues may by molecular mimicry, provide the inducing mechanism for an immune response. The aim of this study was to identify autoantibodies (autoAbs) in nonautoimmune individuals during acute bacterial, viral, or parasitic infections.

Peripheral vascular occlusions leading to gangrene and amputations in antiphospholipid antibody positive patients.

Twenty-one cases from several medical centers (Brazil, Italy, Serbia, South Africa, Israel, and the United Kingdom) with severe peripheral vascular disease progressing to amputations of limbs/digits, all of whom tested positive for antiphospholipid antibodies, are documented. The patients were suffering from either systemic lupus erythematosus, discoid LE, "primary" antiphospholipid syndrome (PAPS), "lupus-like" disease, undifferentiated connective tissue disease. A high frequency of livedo reticularis preceding the arterial occlusions in our series of patients who subsequently progressed to ischemic necrosis and amputation of limbs/digits was noted.

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.

Vascular occlusions in a patient with low positive antiphospholipid antibodies and subsequent development of breast carcinoma: a diagnostic dilemma.

A 61-year-old female with a history of previous digital ischemia and stroke, developed bilateral brachial artery thromboses thought originally to be due to fibromuscular hyperplasia. Histology from one artery revealed bland thrombi only with no evidence of vasculitis. Minimal elevations of anti-B2GP1 were demonstrated with levels of other isotypes measurable at just below cut off levels for normality.

Partial HELLP syndrome in pregnancy complicated by recurrent deep vein thromboses and palmar skin lesions in a patient with prothrombin gene 20210a mutation and antiphospholipid antibodies: an unusual case.

A patient who developed thrombocytopenia and hypertension accompanied by high levels of antiphospholipid antibodies and abnormal liver function tests in the absence of a hemolytic anemia necessitating termination of pregnancy developed bilateral lower limb thromboses accompanied by painful maculo papular lesions on the palms of both hands a few days after ending the pregnancy. She was then also found to have a prothrombin gene G20210A mutation. She was treated with anticoagulation therapy, but her postpartum course was further complicated by pulmonary embolus.

Catastrophic antiphospholipid syndrome: therapeutic developments.

The catastrophic antiphospholipid syndrome is a potentially life-threatening condition with a high mortality rate, the diagnosis of which requires a high degree of clinical awareness on the part of attending physicians. Patients with this syndrome have various symptoms in common: clinical evidence of multiple organ involvement developed over a very short time period, histopathological evidence of multiple small-vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titers. The combination of high doses of intravenous heparin, steroids, gamma-globulins and/or repeated plasma exchanges are the basic treatment of choice for all patients with this severe condition.

Is there a microangiopathic antiphospholipid syndrome?

Revealing the evolution of the term APS and its commonalities with other microangiopathic disorders

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.