Mast cell activation syndrome — clinical presentation and diagnostic criteria

Alergologia ◽  
2021 ◽  
Vol 1 (5) ◽  
pp. 7
Author(s):  
Elena-Simona Boldeanu ◽  
Mădălina Avram ◽  
Lucian Rezmeriţă
Diagnosis ◽  
2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Lawrence B. Afrin ◽  
Mary B. Ackerley ◽  
Linda S. Bluestein ◽  
Joseph H. Brewer ◽  
Jill B. Brook ◽  
...  

AbstractThe concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of “mast cell activation syndrome” (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a “consensus” (re-termed here as “consensus-1”). Another sizable contingent of investigators and practitioners favor a different approach (originally published in 2011, newly termed here as “consensus-2”), resembling “consensus-1” in some respects but differing in others, leading to substantial differences between these proposals in the numbers of patients qualifying for diagnosis (and thus treatment). Overdiagnosis by “consensus-2” criteria has potential to be problematic, but underdiagnosis by “consensus-1” criteria seems the far larger problem given (1) increasing appreciation that MCAS is prevalent (up to 17% of the general population), and (2) most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement. We analyze these proposals (and others) and suggest that, until careful research provides more definitive answers, diagnosis by either proposal is valid, reasonable, and helpful.


2021 ◽  
pp. 135910532110145
Author(s):  
Jennifer Nicoloro SantaBarbara ◽  
Marci Lobel

Individuals with Mast Cell Activation Syndrome (MCAS), a rare chronic disease, experience unpredictable physical symptoms and diagnostic challenges resulting in poor emotional states. The prevalence and correlates of depressive symptoms were examined among 125 participants who completed the CES-D and relevant instruments. The majority reported a clinically-significant level of depression which was especially common among younger participants and those who reported greater loneliness or more disease-specific stressors. Greater magnitude of depressive symptoms was associated with greater illness intrusiveness, less social support, and lower optimism. Results highlight the value of interventions targeting loneliness and stressors unique to this population.


2017 ◽  
Vol 99 (2) ◽  
pp. 190-193 ◽  
Author(s):  
Lawrence B. Afrin ◽  
Roger W. Fox ◽  
Susan L. Zito ◽  
Leo Choe ◽  
Sarah C. Glover

2021 ◽  
Vol 127 (5) ◽  
pp. S49
Author(s):  
M. Hakim ◽  
D. Kurian ◽  
U. Rehman ◽  
S. Kazmi ◽  
A. Ghazi

2021 ◽  
Author(s):  
Isabelle Brock ◽  
Nicole Eng ◽  
Anne Maitland

Abstract Mast Cell Activation syndrome (MCAS) is a clinical condition, defined by the combination of 1) typical symptoms, 2) laboratory abnormalities and 3) response to treatment. Patients present with episodic symptoms of aberrant mast cell activation, such as abdominal cramping, asthma, hypotensive episodes, tachycardia, anaphylaxis, unexplained arrhythmias, and neurologic/psychiatric symptoms. Both clonal and nonclonal mast cell activation syndromes have been described, with a greater prevalence of non-clonal MCAS among the pediatric and adult population. Numerous extrinsic triggers of mast cell activation (MCA) are described, but recent reports point to nonatopic triggers, as the predominant, extrinsic stimulants of MCA in the adult population. The etiology of MCAS is unclear, though recent studies point to the disruption of the epithelium by infection, toxic exposures or physical trauma, and perturbation the tight regulation of these innate immune cells, associated to the epithelial borders. Here we describe a geriatric patient with adult onset MCAS, following a significant toxic exposure, scombroid poisoning. We also review the relevant literature regarding MCAS diagnosis and management as well as potential mechanisms for this hypersensitivity syndrome in adults.


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