McMaster RARE‐Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome

Legault, Kimberly; Schünemann, Holger J.; Hillis, Christopher; Yeung, Cindy H. T.; Akl, Elie A.; Carrier, Marc; Cervera, Ricard; Crowther, Mark; Dentali, Francesco; Erkan, Doruk; Espinosa, Gerard; Khamashta, Munther A.; Meerpohl, Joerg J; Moffat, Karen A.; O’Brien, Sarah H.; Pengo, Vittorio; Rand, Jacob H.; Pintó, Ignasi Rodríguez; Thom, Lorraine; Iorio, Alfonso

doi:10.1111/jth.14192

Cited by 105 publications

(118 citation statements)

References 26 publications

(35 reference statements)

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“…Besides the effectiveness of intervention, the forming of recommendations is based on the severity of the disease, patient willingness, safety, and economics [4]. See Tables 1 and 2 [4,6].…”

Section: Grading the Evidences And Recommendationsmentioning

confidence: 99%

A rapid advice guideline for the diagnosis and treatment of 2019 novel coronavirus (2019-nCoV) infected pneumonia (standard version)

et al. 2020

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In December 2019, a new type viral pneumonia cases occurred in Wuhan, Hubei Province; and then named "2019 novel coronavirus (2019-nCoV)" by the World Health Organization (WHO) on 12 January 2020. For it is a never been experienced respiratory disease before and with infection ability widely and quickly, it attracted the world's attention but without treatment and control manual. For the request from frontline clinicians and public health professionals of 2019-nCoV infected pneumonia management, an evidence-based guideline urgently needs to be developed. Therefore, we drafted this guideline according to the rapid advice guidelines methodology and general rules of WHO guideline development; we also added the first-hand management data of Zhongnan Hospital of Wuhan University. This guideline includes the guideline methodology, epidemiological characteristics, disease screening and population prevention, diagnosis, treatment and control (including traditional Chinese Medicine), nosocomial infection prevention and control, and disease nursing of the 2019-nCoV. Moreover, we also provide a whole process of a successful treatment case of the severe 2019-nCoV infected pneumonia and experience and lessons of hospital rescue for 2019-nCoV infections. This rapid advice guideline is suitable for the first frontline doctors and nurses, managers of hospitals and healthcare sections, community residents, public health persons, relevant researchers, and all person who are interested in the 2019-nCoV.

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Section: Grading the Evidences And Recommendationsmentioning

confidence: 99%

A rapid advice guideline for the diagnosis and treatment of 2019 novel coronavirus (2019-nCoV) infected pneumonia (standard version)

et al. 2020

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“…5,6 Admittedly, the authors comment on "the main challenge in developing this guideline was the low certainty of evidence." 5 Hence, even our best attempt at finding cohesion and helpful treatment recommendations for this malignant benign hematologic disease is bracketed by the absence of certainty. This can be tremendously anxiety-provoking for the physician caring for the patient.…”

Section: Introductionmentioning

confidence: 99%

“…The prevalence of CAPS is unclear but it seems to account for less than 1% of cases of antiphospholipid antibody syndrome . CAPS is classified as a rare disease, defined by the European Union as a condition with a prevalence of less than 1 in 2000 and has a mortality rate as high as 50% . A recent best practice guideline for CAPS highlights the difficulty in making treatment recommendations given insufficient primary evidence, absence of randomized trials, and apprehension about making “weak” recommendations that could pose a barrier to treatment funding .…”

mentioning

confidence: 99%

“…CAPS is classified as a rare disease, defined by the European Union as a condition with a prevalence of less than 1 in 2000 and has a mortality rate as high as 50% . A recent best practice guideline for CAPS highlights the difficulty in making treatment recommendations given insufficient primary evidence, absence of randomized trials, and apprehension about making “weak” recommendations that could pose a barrier to treatment funding . Admittedly, the authors comment on “the main challenge in developing this guideline was the low certainty of evidence.” Hence, even our best attempt at finding cohesion and helpful treatment recommendations for this malignant benign hematologic disease is bracketed by the absence of certainty.…”

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confidence: 99%

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Malignant benign hematology

Sholzberg

2019

Research and Practice in Thrombosis and Haemostasis

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When faced with a life‐threatening non‐cancerous blood disorder, the term “benign” is a misnomer. Devastating diseases like catastrophic antiphospholipid antibody syndrome, acquired hemophilia, and severe immune thrombocytopenia present a challenge to the hematologist. They are often difficult to treat and lack appropriately powered, unbiased evidence to support management. Moreover, the label “benign” does a disservice as it subconsciously triggers discrepancies in prioritization for the care provider, the system, the patient and his/her family. Despite our progressive advances in non‐malignant hematology, there remain many knowledge and care gaps that can be effectively addressed by more international collaboration, more clinical and research infrastructure and more expertly trained clinicians. To highlight the need, is it time to reconsider the term “benign” hematology?

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confidence: 99%

Revisiting the role of therapeutic plasma exchange in the management of catastrophic antiphospholipid syndrome

Peedin

Karp

2019

Journal of Thrombosis and Haemostasis

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As apheresis providers, we are very interested in actively collaborating with multidisciplinary care teams that refer patients for our procedures. We read with interest the recently published McMaster RARE-Bestpractices clinical practice guideline on the diagnosis and management of catastrophic antiphospholipid syndrome (CAPS) [1]. However, the clinical practice guideline had several shortcomings that call into question the authors' conclusion that therapeutic plasma exchange (TPE) should be used as first-line therapy. First, the authors do not cite the "Guidelines on the Use of Therapeutic Apheresis in Clinical Practice" as published by the American Society for Apheresis (ASFA) [2]. The ASFA guidelines are published every 2-3 years and describe the use of apheresis in the management of more than 100 disease processes. These guidelines are supported by a current and comprehensive literature review performed by a group of leading apheresis practitioners. The 2013 and 2016 ASFA guidelines categorize CAPS as a category II indication for TPE, meaning that TPE should be part of second-line therapy. The 2013 ASFA guidelines were available when the McMaster RARE-Bestpractices guideline panel convened in 2016, and the 2016 ASFA guidelines were available during the McMaster RARE-Bestpractices clinical practice guideline public comment period. Additionally, the 2016 ASFA guidelines cite 11 articles concerning the management of CAPS, eight of which are not cited in the McMaster RARE-Bestpractices clinical practice guideline [3-10]. All of these articles were available when the McMaster RARE-Bestpractices guideline panel convened in 2016. Second, the authors misrepresent the risks of TPE. The McMaster RARE-Bestpractices guideline cites an article by Shemin et al. andstates that "common adverse effects of plasmapheresis include fever (7.7%), urticaria (7.4%), and symptomatic hypocalcemia (7.3%)" [1,11].The authors are correct that one of the most common side-effects of TPE is symptomatic hypocalcemia, due to the use of citrate anticoagulation in the apheresis circuit. However, the risk of fever and urticaria is almost entirely dependent on the replacement fluid used during a TPE. As stated by Shemin et al., "Fever, chills, or urticaria (all grouped together) was the most common complication, and this was much more common in patients receiving fresh frozen plasma (FFP) as replacement fluid" [11]. Urticaria occurred in 16.8% of patients receiving FFP as replacement fluid, whereas urticaria occurred in 0.2% of patients receiving albumin/normal saline as replacement fluid [11]. Urticaria and fever are common transfusion reactions; unsurprisingly, these occur when plasma products are used as TPE replacement fluid. Thus, fever and urticaria in the context of TPE are commonly attributable to plasma products, rather than the TPE itself. Further, the authors state that "adverse events occurred in 36% of all patients." This is inaccurate, as Shemin et al. state, "Of the 1727 total treatments, 614 (36%) involved some complication." Thus...

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McMaster RARE‐Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome

Cited by 105 publications

References 26 publications

A rapid advice guideline for the diagnosis and treatment of 2019 novel coronavirus (2019-nCoV) infected pneumonia (standard version)

A rapid advice guideline for the diagnosis and treatment of 2019 novel coronavirus (2019-nCoV) infected pneumonia (standard version)

Malignant benign hematology

Revisiting the role of therapeutic plasma exchange in the management of catastrophic antiphospholipid syndrome

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