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asherson and microangiopathic

Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair Levy
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia...
April 2017: Expert Review of Hematology
Ronald A Asherson, Ricard Cervera, Joan T Merrill, Doruk Erkan
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...
April 2008: Seminars in Thrombosis and Hemostasis
Ronald A Asherson, Ricard Cervera
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. in the case of retinal vascular occlusions). Another group of small vessel occlusions remain unproven and include osteonecrosis, hearing loss and a variety of brain syndromes. All these constitute the microvascular manifestations of the APS...
January 2008: Autoimmunity Reviews
R A Sinico, L Di Toma, E Sabadini, P Renoldi, M Li Vecchi
Catastrophic antiphospholipid syndrome (CAPS), described by Asherson in 1992, is a rare form of antiphospholipid syndrome resulting in multiorgan failure with a mortality rate of about 50%. The syndrome occurs in patients with either systemic lupus erythematosus and other rheumatic diseases (systemic sclerosis, rheumatoid arthritis, primary Sjogren syndrome) or alone. Whereas in "classic" antiphospholipid syndrome (APS), medium-large vessels are involved, a diffuse small vessel ischemia and thrombosis (microangiopathic disease) leading to a severe multiorgan dysfunction is predominant in CAPS...
November 2007: Journal of Nephrology
Ronald A Asherson, Gerard Espinosa, Solomon Menahem, Janet Yinh, Silvia Bucciarelli, Xavier Bosch, Ricard Cervera
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. METHODS: Three patients with relapsing CAPS are presented with their clinical and laboratory features...
June 2008: Seminars in Arthritis and Rheumatism
Ronald A Asherson, Sylvia Pierangeli, Ricard Cervera
No abstract text is available yet for this article.
September 2007: Journal of Rheumatology
I Lazurova, Z Macejova, Z Tomkova, F Remenar, A Boor, J Lazur, R Roland, J Rovensky, R A Asherson
An episode of gastroenteritis triggered severe necrosis of all extremities in a previously asymptomatic male. Hepatic and renal involvement were also manifest, while the hematological picture was one of thrombotic microangiopathic hemolytic anemia. Antiphospholipid antibodies were negative. He responded well to a combination of plasma exchange, anticoagulation (heparin), parenteral steroids, and antibiotics, as well as vasodilators (prostacycline) and hyperbaric oxygen, but died because of a cerebral hemorrhage...
October 2007: Clinical Rheumatology
Ronald A Asherson
The concept of "probable" antiphospholipid syndrome (APS) is almost identical with several conditions which may presage the development of the APS with its major complications of large vessel thromboses resulting in deep vein occlusions in the lower limbs (DVT) particularly and strokes. These conditions comprising livedo reticularis, chorea, thrombocytopenia, fetal loss and valve lesions. These conditions, comprising livedo reticularis, chorea, thrombocytopenia, fetal loss and valve lesions may be followed, often years later by diagnosable APS...
December 2006: Autoimmunity Reviews
G Espinosa, S Bucciarelli, R Cervera, M Lozano, J-C Reverter, G de la Red, V Gil, M Ingelmo, J Font, R A Asherson
OBJECTIVE: To analyse the clinical and laboratory features of patients with thrombotic microangiopathic haemolytic anaemia (TMHA) associated with antiphospholipid antibodies (aPL). METHODS: A computer assisted (PubMed) search of the literature was performed to identify all cases of TMHA associated with aPL from 1983 to December 2002. RESULTS: 46 patients (36 female) with a mean (SD) age at presentation of TMHA of 34 (15) years were reviewed...
June 2004: Annals of the Rheumatic Diseases
R A Asherson, R Cervera
In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagulant tests. The manifestations often include a moderate thrombocytopenia and, less commonly, hemolysis. In contrast, a less frequently encountered subset of the antiphospholipid syndrome, termed the "catastrophic" antiphospholipid syndrome, affects mainly small vessels predominantly supplying organs...
September 2000: Current Opinion in Hematology
R A Asherson
A review of 50 patients who manifest features of the catastrophic antiphospholipid syndrome (CAPS) is presented. The clinical features comprise mainly organ involvement as opposed to large-vessel venous or arterial occlusions as is seen in patients with 'simple' antiphospholipid syndrome (APS), which makes the pathogenesis of this unusually rare complication perhaps somewhat different from that of patients with the APS. The mortality of the condition is 50%, most patients dying as a result of a combination of cardiac and respiratory failure...
1998: Lupus
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