Articles
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). This is a cohort analysis to study the possible association of Ehlers-Danlos syndorm, MCAS and immunodeficiencies. The article highlights the possibility of a correlation and the importance of considering this in EDS patient in order to optimize therapy and prevent tissue damage.
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). This is a brief literature review article which highlights how there may be a connection between Hypermobile Ehlers-Danlos syndrome and MCAS.
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.
Mast cell activation symptoms are prevalent in Long-COVID
Leonard B Weinstock, Jill B Brook, Arthur S Walters, Ashleigh Goris, Lawrence B Afrin, Gerhard J Molderings (2021). This study researched the prevalence of MCAS symptoms post-COVID. The assessment was done through an online questionnaire via a support group. The study observed that MCA symptoms were increased in people with long COVID.
Safe administration of chemotherapy in mast cell activation syndrome
M P Lythgoe, J Krell, I A McNeish, L Tookman (2021). This article details a case report of a 59-year-old MCAS patient with endometrial cancer who received chemotherapy, with the aim to provide a framework for safer chemotherapy administration. It discusses the challenges of administering chemotherapy in patients with MCAS, highlighting hypersensitivity risks of carboplatin and paclitaxel, the role of desensitization protocols, and the need for tailored strategies to safely treat high-risk patients.
Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
Theo Gülen, Cem Akin, Patrizia Bonadonna, Frank Siebenhaar, Sigurd Broesby-Olsen, Knut Brockow, Marek Niedoszytko, Boguslaw Nedoszytko, Hanneke N G Oude Elberink, Joseph H Butterfield, Wolfgang R Sperr, Ivan Alvarez-Twose, Hans-Peter Horny, Karl Sotlar, Juliana Schwaab, Mohamad Jawhar, Roberta Zanotti, Gunnar Nilsson, Jonathan J Lyons, Melody C Carter, Tracy I George, Olivier Hermine, Jason Gotlib, Alberto Orfao, Massimo Triggiani, Andreas Reiter, Karin Hartmann, Mariana Castells, Michel Arock, Lawrence B Schwartz, Dean D Metcalfe, Peter Valent (2021). This article highlights the risk of misdiagnosis or over-diagnosis when applying less stringent criteria for the diagnosis of MCAS. It advocates for a stricter use of the Vienna's consensus criteria for the diagnosis of Mastocytosis and MCAS, in particular the use of tryptase level.
COVID-19 infection in patients with mast cell disorders including mastocytosis does not impact mast cell activation symptoms
Matthew P Giannetti, Emily Weller, Iván Alvarez-Twose, Inés Torrado, Patrizia Bonadonna, Roberta Zanotti, Daniel F Dwyer, Dinah Foer, Cem Akin, Karin Hartmann, Tiago Azenha Rama, Wolfgang R Sperr, Peter Valent, Cristina Teodosio, Alberto Orfao, Mariana Castells (2021). This article highlights how Covid-19 infection in patients with MCAS or mastocytosis does not activate the mastcell nor causes a flare up of mastocytosis symptoms. This finding aligns with the lack of exacerbation of asthma by Covid infection.
Safety of COVID-19 vaccination in patients with mastocytosis and monoclonal mast cell activation syndrome
Rayan Kaakati, Dilawar Khokhar, Cem Akin (2021). This is a retrospective study about the safety of COVID vaccine in patients with different forms of mastocytosis and a history of anaphylaxis. The study highlights that a history of anaphylaxis should not exclude people from vaccination but precautions should be taken to limit the risks (continue current medication, premedication with antihistamine and access to Epipen if normally carried)
mRNA COVID-19 vaccine is well tolerated in patients with cutaneous and systemic mastocytosis with mast cell activation symptoms and anaphylaxis
Tiago Azenha Rama, André Moreira, Mariana Castells (2021). This article is a case report of two individuals with cutaneous and systemic mastocytosis who underwent the COVID-19 vaccination with premedication and did not experience side effects. This article reinforce how understanding mastocytosis and taking the appropriate step can allow safe vaccination practice.
Persistent Antiphospholipid Antibodies, Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome and Post-COVID Syndrome: 1 Year On
Jill R Schofield (2021). This article is a case report about an individual with long COVID that developed POTs, antiphospholid syndrome (APS) and clinical manifestations of MCAS. The individual had potentially mild MCAS symptoms before the Covid infection. The article highlights how treating MCAS, POTs and anti-phospholid with targeted medications can improve clinical manifestations and function. This patient history supports the hypothesis that MCAS could increase the risk of developing dysautonomia following COVID-19 infection.
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