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Background: A variable percentage of patients with systemic mast cell (MC) activation symptoms meet criteria for systemic mastocytosis (SM). We prospectively evaluated the clinical utility of the REMA score versus serum baseline tryptase (sBt) levels for predicting MC clonality and SM in 158 patients with systemic MC activation symptoms in the absence of mastocytosis in the skin (MIS). Methods: World Health Organization criteria for SM were applied in all cases. MC clonality was defined as the presence of KIT-mutated MC or by a clonal HUMARA test. The REMA score consisted of the assignment of positive or negative points as follows: male (+1), female (-1), sBt <15 μg/l (-1) or >25 μg/l (+2), presence (-2) or absence (+1) of pruritus, hives or angioedema and presence (+3) of presyncope or syncope. Efficiency of the REMA score for predicting MC clonality and SM was assessed by receiver operating characteristic (ROC) curve analyses and compared to those obtained by means of sBt levels alone. Results: Molecular studies revealed the presence of clonal MC in 68/80 SM cases and in 11/78 patients who did not meet the criteria for SM. ROC curve analyses confirmed the greater sensitivity and a similar specificity of the REMA score versus sBt levels (84 vs. 59% and 74 vs. 70% for MC clonality and 87 vs. 62% and 73 vs. 71% for SM, respectively). Conclusions: Our results confirm the clinical utility of the REMA score to predict MC clonality and SM in patients suffering from systemic MC activation symptoms without MIS. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Objective: The objective of the present study was to determine the use of systemic corticosteroids (SCs) in patients with bronchial asthma using big data analysis., Methods: We performed an observational, retrospective, noninterventional study based on secondary data captured from free text in the electronic health records. This study was performed based on data from the regional health service of Castille-La Mancha (SESCAM), Spain. We performed the analysis using big data and artificial intelligence via Savana® Manager version 3.0., Results: During the study period, 103 667 patients were diagnosed with and treated for asthma at different care levels. The search was restricted to patients aged 10 to 90 years (mean age, 43.5 [95%CI, 43.4-43.7] years). Of these, 59.8% were women. SCs were taken for treatment of asthma by 58 745 patients at some point during the study period. These patients were older, with a higher prevalence of hypertension, dyslipidemia, diabetes, obesity, depression, and hiatus hernia. SCs are used frequently in the general population with asthma (31.4% in 2015 and 39.6% in 2019). SCs were prescribed mainly in primary care (59%), allergy (13%), and pulmonology (20%). The frequency of prescription of SCs had a direct impact on the main associated adverse effects., Conclusion: In clinical practice, SCs are frequently prescribed to patients with asthma, especially in primary care. Use of SCs is associated with a greater number of adverse events. It is necessary to implement measures to reduce prescription of SCs to patients with asthma, especially in primary care.

Severe asthma has an important impact on patients and healthcare resources. Recently, the new specific treatments have defined a new scenario in which person-focused care and specialist multidisciplinary teams are necessary. Our Severe Asthma Unit (SAU) started the ASfarMA project along with an external human-centered design company to understand patients' vision of their illness, treatment, and healthcare experience, and to define the ideal SAU by performing a core group session, in-depth semistructured interviews and co-creation workshop. Herein, a series of tips classified as either 'transformative solutions' or 'quick wins', according to a value versus effort matrix are presented. Successful implementation of the proposed solutions will be valuable for patients and healthcare professionals, optimising patient care and resources. These findings can also be helpful to other SAUs or other humanisation projects involving complex, chronic and multidisciplinary pathologies., Competing Interests: Competing interests: MMG declares payment or honoraria for lectures, presentations, speakers, bureaus, mansuscript writing for educational events by AstraZeneca and GSK and payment for expert testimony by AstraZeneca. AS-G declares payment or honoraria for lectures, presentations, speakers, bureaus, manuscript writing for educational events by GSK and Sanofi, payment for expert testimony by GSK and Sanofi and support for attending meetings and/or travel by Sanofi. DA-A declares payment or honoraria for lectures, presentations, speakers, bureaus, manuscript writing for educational events by AstraZeneca, GSK, Novartis and Sanofi and payment for expert testimony by AstraZeneca, GSK and Sanofi. Other authors have no conflict of interest to declare., (© European Association of Hospital Pharmacists 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Summary: Anaphylaxis is a potentially fatal hypersensitivity reaction but frequently underrecognized. Although its incidence rates vary according to geographical location, it seems clear that there has been a general increase in recent years, either because of greater recognition of this entity or because it is progressing proportionally to the presence of allergic diseases in the world. The development of anaphylaxis management guidelines adapted to local or regional needs seems of utmost importance. Furthermore, it is necessary to assess their implementation and their positive effect regarding diagnosing and treating anaphylaxis. In this review we explore the currently existing definitions of anaphylaxis and its epidemiology, the potential triggers of anaphylaxis and guideline recommendations in terms of diagnosis and management, proposing a novel anaphylaxis calculator and reviewing the current scoring methods for anaphylactic episodes.

Background: A close association between hereditary alpha-tryptasemia (HAT) and mast cell (MC) disorders has been previously reported. However, the relationship between HAT and the diagnostic subtypes and clinical features of MC disorders still remains to be established., Objective: To determine the prevalence of HAT in healthy donors (HD) vs patients with different diagnostic subtypes of MC activation syndromes (MCAS) and mastocytosis, and its relationship with the clinical behavior of the disease., Methods: A total of 959 subjects were studied including 346 healthy donors (HD), 464 mastocytosis, and 149 non-clonal MCAS patients. Molecular studies to assess the TPSAB1 genotype were performed, and data on serum baseline tryptase (sBT) and basal MC-mediator release episodes and triggers of anaphylaxis were collected., Results: HAT was detected in 15/346 (4%) HD versus 43/149 (29%) non-clonal MCAS and 84/464 (18%) mastocytosis cases. Among mastocytosis, HAT was more frequently found in patients with MC-restricted KIT D816V (21% vs. 10% among multilineage KIT D816V patients; p = .008). Overall, median sBT was higher in cases presenting with HAT (28.9 vs. 24.5 ng/mL; p = .008), while no significant differences in sBT were observed among HAT + mastocytosis patients depending on the presence of 1 vs. ≥2 extra copies of the α-tryptase gene (44.1 vs. 35.2 ng/mL, p > .05). In turn, anaphylaxis was more frequently observed in HAT + versus HAT - mastocytosis patients (76% vs. 65%; p = .018), while HAT + and HAT - patients who did not refer anaphylaxis as the presenting symptom (n = 308) showed a similar prevalence of subsequent anaphylaxis (35% vs. 36%, respectively)., Conclusion: The frequency of HAT in MC disorders varies according to the diagnostic subtype of the disease. HAT does not imply a higher risk (and severity) of anaphylaxis in mastocytosis patients in whom anaphylaxis is not part of the presenting symptoms of the disease., (© 2023 European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)