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Ricard Cervera - One of the best experts on this subject based on the ideXlab platform.
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i11 catastrophic Antiphospholipid Syndrome what is new
Lupus science & medicine, 2020Co-Authors: Ricard CerveraAbstract:Background Catastrophic Antiphospholipid Syndrome (CAPS) is a rare variant that accounts for 1% of patients with Antiphospholipid Syndrome. Methods The current knowledge of this potential devastating entity comes from the International Registry of patients with CAPS, named ‘CAPS Registry’. This presentation shows the results of the most recent analysis of the registry. Results Small vessel thrombosis, laboratory features of microangiopathic haemolytic anemia, and development of multisystem involvement in a very short period of time are the main characteristics of this Syndrome. Clinical manifestations are due to thrombosis but also, although the evidences are indirect, to excess of proinflammatory cytokines. Therefore, treatment strategy is based on the combination of anticoagulation, glucocorticoids, plasma exchange and/or intravenous immunoglobulins, the so-called triple therapy. In refractory cases or in those with initial life-threatening situation, rituximab may be an effective option. Recently, some cases of CAPS have been effectively treated with the addition of eculizumab to the triple therapy. Conclusions Despite its low frequency, the mortality-related is still very high, ranging from 50% of patients in the first series to 37% in the most recent data.
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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, Christopher M Hillis, Cindy H T Yeung, Marc Carrier, Francesco Dentali, Alfonso Iorio, Mark Crowther, Doruk Erkan, Ricard Cervera, 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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the role of infectious diseases in the catastrophic Antiphospholipid Syndrome
Autoimmunity Reviews, 2015Co-Authors: Claudia Mendozapinto, S Maciasdiaz, Vazquez F De Lara, Ivet Etchegaraymorales, Jose Luis Galvezromero, S Mendezmartinez, Mario Garciacarrasco, Ricard CerveraAbstract:Abstract Catastrophic Antiphospholipid Syndrome (CAPS), also called “Asherson Syndrome”, is a variant of the Antiphospholipid Syndrome (APS) that occurs in less than 1% of APS cases. The etiology of CAPS is uncertain; however, several triggering factors have been recognized. The most common of these are infectious diseases, particularly those of the respiratory tract. CAPS pathogenesis is incompletely understood, but several theories have been proposed, such as the molecular mimicry theory, which describes the production of anti-β2-glycoprotein I (GP1) antibody in response to infection. The process is complex and involves the activation of Toll-like receptor 4 (TLR-4), which triggers a cytokine storm, followed by endothelial alterations that induce a procoagulant state.
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the catastrophic Antiphospholipid Syndrome
The Journal of Rheumatology, 2015Co-Authors: I Rodriguezpinto, Gerard Espinosa, Ricard CerveraAbstract:The addition of the word “catastrophic” to the term Antiphospholipid Syndrome (APS) was proposed 20 years ago by Ronald Asherson when he published an editorial in The Journal of Rheumatology describing a group of patients who develop multiple thrombosis in a short period of time and with a much worse prognosis than that attributed to patients with classic APS [1]. Since then, many cases have been published reporting patients with this devastating variant of the APS.
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catastrophic Antiphospholipid Syndrome task force report summary
Lupus, 2014Co-Authors: Ricard Cervera, I RodriguezpintoAbstract:The Task Force on Catastrophic Antiphospholipid Syndrome (CAPS) aimed to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of CAPS. This article summarizes the main aspects of its final report.
Doruk Erkan - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of the Antiphospholipid Syndrome
The New England Journal of Medicine, 2018Co-Authors: David A Garcia, Doruk ErkanAbstract:Antiphospholipid Syndrome Antiphospholipid antibodies can induce endothelial-cell, complement, platelet, neutrophil, and monocyte activation, leading to thrombosis, renal failure, heart valve disea...
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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, Christopher M Hillis, Cindy H T Yeung, Marc Carrier, Francesco Dentali, Alfonso Iorio, Mark Crowther, Doruk Erkan, Ricard Cervera, 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 candidate therapies for a potentially lethal disease
Annual Review of Medicine, 2017Co-Authors: Ozan Unlu, Doruk ErkanAbstract:Catastrophic Antiphospholipid Syndrome (CAPS) is a potentially lethal disease that presents with rapidly progressive multiple organ thromboses. Anticoagulation, corticosteroids, intravenous immunoglobulin, and plasma exchange are the most commonly used treatments for CAPS patients. However, the high mortality despite these medications necessitates new treatment strategies. Following a brief review of current diagnostic and management strategies, we discuss the candidate therapies, i.e., hydroxychloroquine, rituximab, eculizumab, sirolimus, and defibrotide, that can be considered in CAPS patients refractory to traditional treatment.
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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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aps action Antiphospholipid Syndrome alliance for clinical trials and international networking
Lupus, 2012Co-Authors: Doruk ErkanAbstract:Antiphospholipid Syndrome Alliance For Clinical Trials and InternatiOnal Networking (APS ACTION) is the first-ever international research network that has been created specifically to design and conduct well-designed, large-scale, multi-center clinical trials in persistently Antiphospholipid antibody (aPL)-positive patients. The founding principle of the APS ACTION is that it is an internationally collaborative effort, open to qualified investigators across the globe who are committed to furthering our understanding of APS and its management. Due to the hard work and collaborative spirit of APS ACTION members, in early 2012, APS ACTION launched two important collaborative international projects: 1) a randomized controlled trial of hydroxychloroquine in the primary thrombosis prevention of persistently aPL-positive but thrombosis-free patients without other systemic autoimmune diseases; and 2) a web-based registry of aPL-positive patients with or without systemic autoimmune diseases, which will also include annual blood collection for aPL-testing and future basic science studies. In the end, we hope to find better treatments for Antiphospholipid Syndrome, which is a leading cause of thrombosis, pregnancy morbidity and other life-altering consequences, and to heighten awareness about this life-threatening, autoimmune condition. Lupus (2012) 21, 695–698.
Ronald A Asherson - One of the best experts on this subject based on the ideXlab platform.
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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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relapsing catastrophic Antiphospholipid Syndrome report of three cases
Seminars in Arthritis and Rheumatism, 2008Co-Authors: Ronald A Asherson, Gerard Espinosa, Solomon Menahem, Janet Yinh, Silvia Bucciarelli, Xavier Bosch, Ricard CerveraAbstract: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. Results Seven episodes of CAPS that occurred in the 3 patients reported were analyzed. The median time between the episodes of CAPS was 12.5 months (range, 2.5-48). Precipitating factors were identified in 2 episodes only ( Legionella respiratory tract infection and periodontal infection). The most significant manifestations of the episodes were renal involvement (5 episodes), central nervous system and cardiac involvement (4 episodes), and pulmonary and hepatic involvement (3 episodes each). Interestingly, laboratory features of definite microangiopathic hemolytic anemia (MHA) were present in 5 of 7 episodes of relapsing CAPS. The remaining episodes presented with thrombocytopenia, schistocytes, and anemia but data concerning hemolysis and Coombs tests were not reported. Rituximab was used in 2 episodes. Conclusions Relapses occur very infrequently in patients with the CAPS. The presence of MHA is common in these patients, suggesting that an association between MHA and relapses of CAPS could be present and that a "continuum" between various MHAs might exist, as recently suggested.
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catastrophic Antiphospholipid Syndrome
Rheumatic Diseases Clinics of North America, 2006Co-Authors: Ricard Cervera, Ronald A Asherson, Juli 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
Journal of Intensive Care Medicine, 2006Co-Authors: Setu 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
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.
Gerard Espinosa - One of the best experts on this subject based on the ideXlab platform.
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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, Christopher M Hillis, Cindy H T Yeung, Marc Carrier, Francesco Dentali, Alfonso Iorio, Mark Crowther, Doruk Erkan, Ricard Cervera, 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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the catastrophic Antiphospholipid Syndrome
The Journal of Rheumatology, 2015Co-Authors: I Rodriguezpinto, Gerard Espinosa, Ricard CerveraAbstract:The addition of the word “catastrophic” to the term Antiphospholipid Syndrome (APS) was proposed 20 years ago by Ronald Asherson when he published an editorial in The Journal of Rheumatology describing a group of patients who develop multiple thrombosis in a short period of time and with a much worse prognosis than that attributed to patients with classic APS [1]. Since then, many cases have been published reporting patients with this devastating variant of the APS.
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update on the catastrophic Antiphospholipid Syndrome and the caps registry
Seminars in Thrombosis and Hemostasis, 2012Co-Authors: Ricard Cervera, Gerard EspinosaAbstract:Although less than 1% of patients with the Antiphospholipid Syndrome (APS) develop the catastrophic variant known as catastrophic Antiphospholipid Syndrome (CAPS), 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. To collate all the published case reports as well as the newly diagnosed cases from all over the world, an international registry of patients with CAPS (“CAPS Registry”) was created in 2000 by the European Forum on Antiphospholipid Antibodies ( www.med.ub.es/MIMMUN/FORUM/CAPS.HTM ). Currently, this database documents the entire clinical, laboratory, and therapeutic data of more than 350 fully registered patients.
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management of refractory cases of catastrophic Antiphospholipid Syndrome
Autoimmunity Reviews, 2011Co-Authors: Gerard Espinosa, Horacio Berman, Ricard CerveraAbstract:Abstract The catastrophic variant of the Antiphospholipid Syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. Refractory catastrophic APS may be defined as patients who did not respond to first-line therapies (anticoagulation, glucocorticoids and plasma exchange and/or intravenous immunoglobulins) and died in the acute phase of the episode or patients with recurrent episodes of catastrophic APS. The purpose of this review is to focus on the current management of these refractory patients and some of the potential new therapeutic approaches.
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catastrophic Antiphospholipid Syndrome updated diagnostic algorithms
Autoimmunity Reviews, 2010Co-Authors: Doruk Erkan, Gerard Espinosa, Ricard CerveraAbstract:Abstract The catastrophic Antiphospholipid Syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. During the 13th International Congress on Antiphospholipid Antibodies (aPL) a “Catastrophic APS Task Force” was developed to discuss the challenges in the diagnosis and management of catastrophic APS. The purpose of this paper is to summarize the diagnostic challenges (false-positive/negative aPL results and overlap with other thrombotic microangiopathies) and propose updated diagnostic algorithms for catastrophic APS. Important steps of the diagnostic algorithms include: a) history of APS or persistent aPL-positivity; b) three or more organ new thrombosis developing in less than a week; c) biopsy diagnosis of microthrombosis; and d) other explanations for multiple organ thromboses and/or microthrombosis.
M C Freiredapena - One of the best experts on this subject based on the ideXlab platform.
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livedoid vasculopathy and recurrent thrombosis in a patient with lupus seronegative Antiphospholipid Syndrome
Lupus, 2010Co-Authors: B Sopena, A Rivera, M T Perezrodriguez, J A Ortizrey, Jose Luis Lamas, M C FreiredapenaAbstract:Livedoid vasculopathy is a rare condition which predominantly affects young women. It is characterized by intense painful purpuric maculae in the legs, ankles and feet, due to thrombosis of the small and medium-sized dermal vessels, in the absence of vasculitis. Livedoid vasculopathy has been frequently associated with hypercoagulable states and Antiphospholipid Syndrome. We describe a 34-year-old White woman suffering from systemic lupus erythematosus, livedo reticularis, haemolytic anaemia, severe thrombocytopenia and recurrent venous thrombosis who was admitted to the hospital for extremely painful purpuric lesions in her lower limbs. The clinical and histological findings were diagnostic of livedoid vasculopathy. Once the initial sub-therapeutic international normalized ratio levels were corrected, livedoid vasculopathy did not recur. Tests for Antiphospholipid antibodies were repeatedly negative. This case, the first reported of livedoid vasculopathy in a patient with seronegative Antiphospholipid Syndrome and systemic lupus erythematosus, draws attention to livedoid vasculopathy, a thrombotic dermopathy that may be under-diagnosed in patients with Antiphospholipid Syndrome.