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Ricard Cervera - One of the best experts on this subject based on the ideXlab platform.
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Catastrophic Antiphospholipid Syndrome: therapeutic developments.
Expert Review of Clinical Immunology, 2020Co-Authors: Ricard Cervera, Ronald A AshersonAbstract: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, γ-globulins and/or repeated plasma exchanges are the basic treatment of choice for all patients with this severe condition.
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Susac's Syndrome or Catastrophic Antiphospholipid Syndrome?
Lupus, 2020Co-Authors: Silvia Bucciarelli, Ricard Cervera, Miguel Molina Martínez, X Latorre, J FontAbstract:Susac’s Syndrome is a microangiopathic disorder of unknown pathogenesis presenting with encephalopathy, hearing loss and branch retinal artery occlusions. The term ‘Catastrophic’ Antiphospholipid Syndrome (APS) is used to define a subset of the APS characterized by thrombotic microangiopathy with clinical evidence of three or more organ involvement developed in a short period of time. We describe a patient with typical features of Susac’s Syndrome, that appeared in less than a week, in whom aPL were detected, thus fulfilling criteria for ‘probable’ Catastrophic APS.
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Chapter 17 - Treatment of Catastrophic Antiphospholipid Syndrome
Handbook of Systemic Autoimmune Diseases, 2020Co-Authors: Ignasi Rodríguez-pintó, Gerard Espinosa, Ricard CerveraAbstract:Abstract Current guidelines recommend triple therapy with anticoagulation (AC), glucocorticoids (GC), plasma exchange (PE), and/or intravenous immunoglobulins (IVIG) as the specific therapy for Catastrophic Antiphospholipid Syndrome (CAPS) patients. Of note, only AC had shown significant effect in improving the vital prognosis of these patients. However, there is indirect evidence to advocate the use of other immunomodulatory therapies together with AC (ie, GC, PE, and IVIG) in CAPS, providing a multimodal therapy available to hamper further organ dysfunction due to multiorgan thrombosis and following cytokine storm due to tissue necrosis. Recently, two new monoclonal antibodies (rituximab and eculizumab) seem to have become a good complement to the standard therapy. The first one binds to a protein expressed on the cell membrane of B cells, thus avoiding their development and the evolution to autoantibodies-generating plasma cells. The second one hampers complement pathway activation by binding to the C5 and inhibiting its cleavage. Thus, it precludes the chemoattractant C5a generation and prevents membrane attack complex generation.
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thu0561 clinical practice guideline for diagnosis and management of Catastrophic Antiphospholipid Syndrome
Annals of the Rheumatic Diseases, 2017Co-Authors: Kimberly Legault, Doruk Erkan, Ricard Cervera, Christopher M Hillis, Cindy H T Yeung, Alfonso Iorio, Mark Crowther, Marc Carrier, Francesco Dentali, Gerard EspinosaAbstract:Background Catastrophic Antiphospholipid Syndrome (CAPS) is characterized by the rapid onset of widespread or multifocal large and/or small vessel thrombosis associated with multi-organ failure in patients meeting the serological criteria for Antiphospholipid Syndrome [1]. Mortality in CAPS approaches 50% [2]. Objectives The RARE-BestPractices project group identified CAPS as a rare disease condition of interest in which to develop a clinical practice guideline. The project was run in partnership with McMaster University, and used the GIN-McMaster Guideline Development checklist and Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology to develop guidelines on rare diseases [3]. Methods The CAPS guideline was coordinated by a steering committee including representatives from RARE-BP and methodologists from McMaster University. The CAPS guideline panel consisted of 19 international members, including patient representation. The panel used the GradePro software to brainstorm and prioritize potential questions and outcomes. Systematic reviews were performed for each question. To supplement the published evidence, we compiled raw data for mortality from the CAPS Registry, and systematically elicited expert opinion from the panel members using a systematic observation form. For each question an evidence profile and evidence to decision table was generated and shared. Results The question prioritization step generated 47 questions, which were ranked to identify the top priorities. The top 10 questions were chosen for guideline development, yielding 7 therapy and 3 diagnostic questions. The outcome generation step yielded 7 outcomes. The questions were addressed during an in-person panel meeting, held on April 27, 2016 in Barcelona, Spain, with follow-up via webinar on June 3, 2016, and with web-based voting completed July 31, 2016. Recommendations were developed for all questions and will be discussed in detail. Conclusions Ten recommendations were issued by the CAPS Guideline Panel to assist clinicians in diagnosis and management of suspected CAPS patients. Future research is needed to improve evidence quality in rare diseases such as CAPS. The GIN-McMaster Guideline Development Checklist and the GRADE methodology were effective in producing a rigorous guideline in this rare disease. References Asherson R. The Catastrophic Antiphospholipid Syndrome. J Rheumatol 1992. Cervera R, Bucciarelli S, Plasin M, et al. Catastrophic Antiphospholipid Syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32:240–5. Guyatt G, Oxman A, Vist G, et al. GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 2008;336:924–6. Disclosure of Interest None declared
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Catastrophic Antiphospholipid Syndrome the current management approach
Best Practice & Research: Clinical Rheumatology, 2016Co-Authors: I Rodriguezpinto, Gerard Espinosa, Ricard CerveraAbstract:Abstract The current recommendation for Catastrophic Antiphospholipid Syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only AC has a significant effect on the prognosis of these patients. However, from the experimental or basic point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies (GC, PE, and IVIG) in CAPS. Recently, there have been reports of severe or refractory CAPS patients treated with the monoclonal antibodies rituximab and eculizumab. The first blocks CD20, a surface protein expressed on the cytoplasmic membrane of B cells, and decreases the generation of pathogenic autoantibodies such as Antiphospholipid (aPL) antibodies. The second binds with high affinity to C5 complement protein, inhibiting its cleavage and thus preventing the generation of C5b–C9 complex.
J Font - One of the best experts on this subject based on the ideXlab platform.
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Susac's Syndrome or Catastrophic Antiphospholipid Syndrome?
Lupus, 2020Co-Authors: Silvia Bucciarelli, Ricard Cervera, Miguel Molina Martínez, X Latorre, J FontAbstract:Susac’s Syndrome is a microangiopathic disorder of unknown pathogenesis presenting with encephalopathy, hearing loss and branch retinal artery occlusions. The term ‘Catastrophic’ Antiphospholipid Syndrome (APS) is used to define a subset of the APS characterized by thrombotic microangiopathy with clinical evidence of three or more organ involvement developed in a short period of time. We describe a patient with typical features of Susac’s Syndrome, that appeared in less than a week, in whom aPL were detected, thus fulfilling criteria for ‘probable’ Catastrophic APS.
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Catastrophic Antiphospholipid Syndrome
Rheumatic Diseases Clinics of North America, 2006Co-Authors: Ricard Cervera, Ronald A Asherson, J FontAbstract:The Catastrophic Antiphospholipid Syndrome is a potentially life-threatening condition with a high mortality, which requires a high degree of clinical awareness on the part of attending physicians. Patients with this Syndrome have in common: clinical evidence of multiple organ involvement developed over a very short time period; histopathologic evidence of multiple small vessel occlusions; and laboratory confirmation of the presence of Antiphospholipid antibodies, usually in high titer. The combination of high doses of intravenous (iv) heparin, iv steroids, iv gammaglobulins, or repeated plasma exchanges is the basic treatment of choice for all patients with this severe condition.
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Catastrophic Antiphospholipid Syndrome clinical description and triggering factors in 8 patients
The Journal of Rheumatology, 2000Co-Authors: Jorge Rojasrodriguez, Ricard Cervera, M Garciacarrasco, Manuel Ramoscasals, G Enriquezcoronel, C Colchero, J FontAbstract:In 1992, an attempt to single out a different and important group of patients was adopted by introducing the term Catastrophic' Antiphospholipid Syndrome (APS) to describe their potentially life threatening clinical course, characterized by acute multiorgan failure (3 or more organ systems damaged). Patients previously described in the literature showed certain differences that appear to distinguish this minority group of patients with Catastrophic APS from the majority of APS patients. We describe 8 patients with Catastrophic APS, focusing especially on the possible extrinsic factors (mainly infections) that might trigger this Catastrophic situation.
Andrea Lavinio - One of the best experts on this subject based on the ideXlab platform.
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probable Catastrophic Antiphospholipid Syndrome with intracerebral hemorrhage secondary to epstein barr viral infection
Neurocritical Care, 2018Co-Authors: Mark Plummer, Adam Young, Ronan Oleary, Maxwell S Damian, Andrea LavinioAbstract:Background Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, severe variant of Antiphospholipid Syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein–Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management.
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Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein–Barr Viral Infection
Neurocritical Care, 2017Co-Authors: Mark P. Plummer, Adam Young, Maxwell S Damian, Ronan O’leary, Andrea LavinioAbstract:Background Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, severe variant of Antiphospholipid Syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein–Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management.
Yehuda Shoenfeld - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of Catastrophic Antiphospholipid Syndrome
Expert Review of Hematology, 2017Co-Authors: Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair LevyAbstract:ABSTRACTIntroduction: 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.Expert commentary: CAPS is a rare variant of the Antiphospholipid Syndrome (APS). It is characterized by thrombosis in multiple organs and a cytokine storm developing over a short period, with histopathologic evidence of multiple microthromboses, and laborator...
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14th international congress on Antiphospholipid antibodies task force report on Catastrophic Antiphospholipid Syndrome
Autoimmunity Reviews, 2014Co-Authors: Ricard Cervera, Yehuda Shoenfeld, I Rodriguezpinto, Serena Colafrancesco, Fabrizio Conti, Guido Valesini, Cristina Rosario, Nancy AgmonlevinAbstract:The 'Task Force on Catastrophic Antiphospholipid Syndrome (CAPS)' was developed on the occasion of the 14th International Congress on Antiphospholipid Antibodies. The objectives of this Task Force were to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of this condition in order to address recommendations for future research. This article summarizes the studies analyzed by the Task Force, its recommendations and the future research agenda.
Ronald A Asherson - One of the best experts on this subject based on the ideXlab platform.
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Catastrophic Antiphospholipid Syndrome.
Journal of intensive care medicine, 2020Co-Authors: Setu K Vora, Ronald A Asherson, Doruk ErkanAbstract:Catastrophic Antiphospholipid Syndrome is a rapidly progressive life-threatening disease that causes multiple organ thromboses and dysfunction in the presence of Antiphospholipid antibodies. A high index of clinical suspicion and careful investigation are required to make an early diagnosis so that treatment with anticoagulation and corticosteroids can be initiated; plasma exchange and/or intravenous immunoglobulins can be added if the life-threatening condition persists. Despite aggressive treatment and intensive care unit management, patients with Catastrophic Antiphospholipid Syndrome have a 48% mortality rate, primarily attributable to cardiopulmonary failure. This article reviews the current information on the etiopathogenesis, clinical manifestations, diagnosis, management, and prognosis of Catastrophic Antiphospholipid Syndrome.
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Catastrophic Antiphospholipid Syndrome: therapeutic developments.
Expert Review of Clinical Immunology, 2020Co-Authors: Ricard Cervera, Ronald A AshersonAbstract: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, γ-globulins and/or repeated plasma exchanges are the basic treatment of choice for all patients with this severe condition.
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Catastrophic Antiphospholipid Syndrome
Case reports in rheumatology, 2016Co-Authors: Setu Vora, Ronald A Asherson, Doruk ErkanAbstract:This paper reports one case of successfully treated patients suffering from a rare entity, the Catastrophic Antiphospholipid Syndrome (CAPS). Management of this patient is discussed in detail.
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Catastrophic Antiphospholipid Syndrome
Rheumatic Diseases Clinics of North America, 2006Co-Authors: Ricard Cervera, Ronald A Asherson, J FontAbstract:The Catastrophic Antiphospholipid Syndrome is a potentially life-threatening condition with a high mortality, which requires a high degree of clinical awareness on the part of attending physicians. Patients with this Syndrome have in common: clinical evidence of multiple organ involvement developed over a very short time period; histopathologic evidence of multiple small vessel occlusions; and laboratory confirmation of the presence of Antiphospholipid antibodies, usually in high titer. The combination of high doses of intravenous (iv) heparin, iv steroids, iv gammaglobulins, or repeated plasma exchanges is the basic treatment of choice for all patients with this severe condition.
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Catastrophic Antiphospholipid Syndrome
Nature Reviews Rheumatology, 2006Co-Authors: Joan T Merrill, Ronald A AshersonAbstract:Catastrophic Antiphospholipid Syndrome, a severe form of Antiphospholipid Syndrome, is associated with a high mortality rate; approximately 50% of patients die from thrombotic diathesis. As discussed in this Review, efforts to facilitate early diagnosis, institute effective treatments in a timely manner, and to better understand the causes of this extreme condition are needed to improve patient outcome.