Articles
Learned cautions regarding antibody testing in mast cell activation syndrome
Lawrence B. Afrin, Tania T. Dempsey and Gerhard J. Molderings (2023). This article discusses the role and limitations of antibody testing in MCAS patients. As MCAS causes abnormal antibody production, it can mimic antibodies that can lead to diagnostic confusion and misdiagnosis of autoimmune or infectious diseases. The article suggests that healthcare professionals should exercise caution with positive antibody test results in MCAS patients. It states that the monitoring of clinical symptoms and repeated testing over time should be used to avoid misdiagnosis caused by misleading results.
Mast Cells as a Target - A Comprehensive Review of Recent Therapeutic Approaches
Joanna Baran, Anna Sobiepanek, Anna Mazurkiewicz-Pisarek, Marta Rogalska, Aleksander Gryciuk, Lukasz Kuryk, Soman N Abraham, Monika Staniszewska (2023). This article provides a comprehensive overview of therapeutic approaches targeting mast cells, making it highly relevant to the treatment theme. It discusses the role of mast cells in the immune system, their overactivity in pathological states, and various therapeutic strategies to manage these conditions.
Immunological dysfunction and mast cell activation syndrome in long COVID
Stevent Sumantri, Iris Rengganis (2023). This article explores the similarities between long COVID and MCAS; long COVID-19 is persistent inflammatory state that causes abnormal mast cell activation and the release of inflammatory cytokines. This connection opens therapeutic opportunities for patients with long COVID that have clinical symptoms consistent with MCAS.
How can a dietician help support those with Mast Cell Activation Syndrome?
Chloe Hall (2023). This article gives an overview of dietary triggers associated with MCAS and dietary recommendations for the management of MCAS symptoms. It is targeted to dieticians, outlining their role in identifying dietary triggers in MCAS, safely re-introducing foods where anaphylaxis has occurred in the past, and in fostering a healthy relationship between MCAS patients and food.
KITD816V mutation in blood for the diagnostic screening of systemic mastocytosis and mast cell activation syndromes
Paula Navarro-Navarro, Iván Álvarez-Twose, Alba Pérez-Pons, Ana Henriques, Andrea Mayado, Andrés C. García-Montero, Laura Sánchez-Muñoz, Oscar González-López, Almudena Matito, Carolina Caldas, María Jara-Acevedo, Alberto Orfao (2022). This article discusses the potential utility of investigating KITD816V in genomic DNA to increase diagnostic sensitivity for MCAS. It states that, while KITD816V is a key marker for systemic mastocytosis, it is less detectable in blood for patients with low levels of the mutation, such as patients with c-MCAS. It suggests that tracking the KITD816V mutation over time could help identify patients at higher risk of disease progression.
Mast Cell Diseases in Practice and Research: Issues and Perspectives Raised by Patients and Their Recommendations to the Scientific Community and Beyond
Susan V. Jennings, Celeste C. Finnerty, Jessica S. Hobart, Mercedes Martín-Martínez, Kristin A. Sinclairf, Valerie M. Slee, Julie Agopian, Cem Akin, Ivan Álvarez-Twose, Patrizia Bonadonna, Angela S. Bowman, Knut Brockow, Horia Bumbea, Claudia de Haroo, Jie Shen Fok, Karin Hartmann, Nicole Hegmanns, Olivier Hermine, Monika Kalisiak, Constance H. Katelaris, Jacqueline Kurzs, Patrizia Marcis, David Mayne, David Mendoza, Alain Moussy, Genija Mudretzkyjz, Nicoleta Nidelea Vaiaaa, Marek Niedoszytko, Hanneke Oude Elberink, Alberto Orfao, Deepti H. Radia, Sophie Rosenmeierz ∙ Eugenia Ribadad ∙ Waltraud Schinhofenff ∙ Juliana Schwaab, Frank Siebenhaar, Massimo Triggiani, Giuseppe Tripodox, Rocio Velazquezo, Yvon Wielinkkk, Friedrich Wimazal, Timo Yigitff, Celia Zubrinich, Peter Valent (2022).This article focuses on the perspectives of those with mast cell diseases (namely mastocytosis and MCAS). It details a project aiming to expand our understanding of patient needs and to propose actions that address those needs. Issues reported include struggles relating to the rarity of mast cell diseases, the impact of the diseases on quality of life, the diagnostic process and the need for further research. It also identifies possible solutions to these challenges.
Mast Cells and Vitamin D Status: A Clinical and Biological Link in the Onset of Allergy and Bone Diseases
Giuseppe Murdaca, Alessandro Allegra, Alessandro Tonacci, Caterina Musolino, Luisa Ricciardi, Sebastiano Gangemi (2022). This article analyses the relationships between mast cells and vitamin D, highlighting their roles in allergic diseases, bone metabolism, and skin conditions. It discusses how vitamin D influences mast cell activation, degranulation, and pro-inflammatory mediator release. Therefore, the findings suggest that vitamin D is a potential therapeutic strategy for managing mast cell driven conditions such as MCAS.
Depression, psychosocial correlates, and psychosocial resources in individuals with mast cell activation syndrome
Jennifer Nicoloro SantaBarbara, Marci Lobel (2022). This article examines the psychological and emotional experiences of 125 participants with MCAS. Clinical depression was found to be common in the sample (64% prevalence), as well as feelings of loneliness and defectiveness, which contributed to high illness intrusiveness. The study concludes that while stigma and self-efficacy overlap with other variables, loneliness and disease-specific stressors emerge as significant risks, and optimism acts as a resilience factor.
Mast cell activation and the link with long COVID
Soumya Arun, Abbie Storan, Bethan Myers (2022). This article provides a comprehensive overview of MCAS biomarkers, diagnosis and management before relating the disease to long COVID. It discusses the overlap in symptoms, the potential exacerbation or incitement of MCAS by COVID-19 infection, common environmental triggers, and similarities in relapse-remission cycles in people with long COVID and people with MCAS. It also discusses improvements with similar medications in both diseases, but emphasises that despite these similarities, other underlying pathologies may also be associated with long COVID aside from mast cell disorders.
Health-related quality of life and health literacy in patients with systemic mastocytosis and mast cell activation syndrome
Tobias Jürgen Schmidt, Julia Sellin, Gerhard J Molderings, Rupert Conrad, Martin Mücke (2022). This article assesses the health-related quality of life (HRQOL) and health literacy of people suffering from Systemic Mastocytosis (SM) and Mast Cell Activation Syndrome (MCAS). It finds that SM and MCAS have a significant impact on the quality of life of individuals, and patients with SM typically receive significantly more information from physicians about their disease. Their results demonstrate the importance of patients’ level of information, as even slight improvements in health literacy can have a positive effect on their quality of life.
Drug-induced mast cell eradication: A novel approach to treat mast cell activation disorders?
Peter Valent, Cem Akin, Karin Hartmann, Andreas Reiter, Jason Gotlib, Karl Sotlar, Wolfgang R. Sperr, Lina Degenfeld-Schonburg, Dubravka Smiljkovic, Massimo Triggiani, Hans-Peter Horny, Michel Arock, Stephen J. Galli, Dean D. Metcalfe (2022). This article examines the potential of KIT-targeting tyrosine kinase inhibitors (TKIs) in treating MCAS by blocking mast cell activation. It highlights that while TKIs like avapritinib show promise in targeting KIT D816V-positive mast cells, their effectiveness in MCAS remains unclear. The authors suggest more research is needed to determine the long-term safety and efficacy of these treatments for MCAS patients.
Mast Cell Activation Syndrome in COVID-19 and Female Reproductive Function: Theoretical Background vs. Accumulating Clinical Evidence
Dariusz Szukiewicz, Piotr Wojdasiewicz, Mateusz Watroba, Grzegorz Szewczyk (2022). This article discusses the importance of mast cells in the female reproductive system and discusses the mechanism of potential disorders related to MCAS. While it suggests that COVID-19 hyperinflammation may involve MCAS, it finds that current evidence does not indicate a significant impact on female reproductive function
Mast cell activation syndrome - anesthetic challenges in two different clinical scenarios
Brianna Lide, Shane McGuire, Hong Liu, Cristina Chandler (2022). This is a case report outlining two clinical scenarios of MCAS, aiming to identify possible factors triggering mast cell mediator release. It discusses diagnosis criteria, symptoms, triggers, treatment, premedication and anaphylaxis. It concludes that recognizing and managing MCAS with trigger avoidance and tailored treatment plans is essential for reducing morbidity and achieving optimal outcomes.
Idiopathic mast cell activation syndrome is more often suspected than diagnosed - A prospective real-life study
Thomas Buttgereit, Sophie Gu, Leonor Carneiro-Leão, Annika Gutsche, Marcus Maurer, Frank Siebenhaar (2022). This article details a prospective study of 100 patients with suspected idiopathic MCAS. It investigates the prevalence of diagnostic criteria including increased tryptase and response to MC-targeted treatment. It finds that only 2 of 79 patients had increased tryptase following an episode, and that depression and anxiety disorders were frequent comorbidities. It concludes that, as MCAS was confirmed in only 2% of patients, it is not MC activation that drives symptoms in most patients with suspected MCAS. It calls for further research on the pathogenesis of the disease.
High FODMAP diet causes barrier loss via lipopolysaccharide-mediated mast cell activation
Prashant Singh, Gintautas Grabauskas, Shi-Yi Zhou, Jun Gao, Yawen Zhang, Chung Owyang (2021). This article discusses how a high FODMAP (HFM) diet induces mast cell activation and colonic barrier dysfunction in rodent models and patients with IBS-D. It highlights the critical role of TLR4-dependent mast cell activation in FODMAP-induced barrier loss and the potential benefits of a low FODMAP (LFM) diet in reducing mast cell activation and improving colonic barrier function. The study provides new insights into the microbiome’s contribution to mast cell activation and its relevance for managing gastroinstestinal symptoms in IBS-D.
Mast cell activation disease and immunoglobulin deficiency in patients with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder
Isabelle Brock, Walter Prendergast, Anne Maitland (2021).
Mast cell activation may explain many cases of chemical intolerance
Claudia S. Miller, Raymond F. Palmer, Tania T. Dempsey, Nicholas A. Ashford & Lawrence B. Afrin (2021). This article discusses the similarities between Mast Cell Activation Syndrome (MCAS) and Toxicant-Induced Loss of Tolerance (TILT), suggesting that both conditions share overlapping symptoms due to mast cell activation. It proposes that MCAS may provide a biological mechanism for TILT, with both conditions exhibiting multi-system symptoms triggered by environmental exposures. The article concludes that more research should be done to improve our understanding of the connection between TILT and MCs, and of new links between environmental exposures and illness.
Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal
Peter Valent, Cem Akin, Karin Hartmann, Ivan Alvarez-Twose, Knut Brockow, Olivier Hermine, Marek Niedoszytko, Juliana Schwaab, Jonathan J Lyons, Melody C Carter, Hanneke Oude Elberink, Joseph H Butterfield, Tracy I George, Georg Greiner, Celalettin Ustun, Patrizia Bonadonna, Karl Sotlar, Gunnar Nilsson, Mohamad Jawhar, Frank Siebenhaar, Sigurd Broesby-Olsen, Selim Yavuz, Roberta Zanotti, Magdalena Lange, Boguslaw Nedoszytko, Gregor Hoermann, Mariana Castells, Deepti H Radia, Javier I Muñoz-Gonzalez, Wolfgang R Sperr, Massimo Triggiani, Hanneke C Kluin-Nelemans, Stephen J Galli, Lawrence B Schwartz, Andreas Reiter, Alberto Orfao, Jason Gotlib, Michel Arock, Hans-Peter Horny, Dean D Metcalfe (2021). This article discusses Mastocytosis, and its classification into cutaneous, systemic, and MC sarcoma forms. It highlights the role of the KIT D816V mutation and other genetic markers in determining disease severity and prognosis. The article also proposes updated diagnostic criteria and a global classification system of MC disorders generally, including MCAS.
Some cases of hypermobile Ehlers-Danlos syndrome may be rooted in mast cell activation syndrome
Lawrence B Afrin (2021).
Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
Ritsuko Kohno, David S Cannom, Brian Olshansky, Shijun Cindy Xi, Darshan Krishnappa, Wayne O Adkisson, Faye L Norby, Artur Fedorowski, David G Benditt (2021). This study examines the frequency with which findings suggesting mast cell activation (MCA) disorder occurred in patients diagnosed with postural orthostatic tachycardia syndrome (POTS). It notes a broader symptom profile in MCA cases, including allergic and gastrointestinal issues. It identifies prostaglandins combined with other markers, such as histamine, as useful but not definitive diagnostic indicators and concludes that MCA should be considered in POTS-like cases with atypical symptoms.
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