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Selective IgM deficiency (SIgMD) has recently been included in the inborn errors of immunity classification. SIgMD has conflicting diagnostic criteria and diverse clinical and immunological findings. We aimed to assess the clinical and laboratory profiles of patients with SIgMD and to compare the data of patients diagnosed using two inclusion criteria. This was a descriptive, retrospective, observational, collaborative study. Patients were included according to the following definitions: Group 1, IgM levels < 0.20 g/L in children and < 0.30 g/L in adults, and Group 2, serum IgM levels below 2SD and, for both, absence of associated immunological diseases or secondary causes. The protocol was approved by the Ethics Committee, and patients provided consent. In total, 75 patients were included: 37 (16 M:21F; mean age, 52.92) and 38 (13 M:25F; mean age, 53.47) in Groups 1 and 2, respectively. The most frequent clinical manifestations were allergic rhinitis (G1, 45.9%; G2, 36.8%), asthma (G1, 37.8%; G2, 28.9%), and pulmonary infections (G1, 27.03%; G2, 21.05%). Chromosomopathies (16.22%) and neoplasia (13.51%) were more frequent in G1, whereas URTI (23.68%) and skin infections (23.68%) were more common in G2. There was no difference in sex or mean age at symptom onset between both groups of patients. Regarding the clinical picture, 90.7% of the lesions were benign (68/75). Chromosomopathies may be associated with SIgMD, suggesting the need to quantify serum IgM levels in these cases. Considering the possibility of developing autoimmunity, neoplasia, and common variable immunodeficiency, it is advisable to follow up patients with SIgMD., Competing Interests: Declarations. Ethics approval: This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of University Center FMABC on January 17, 2021 (CAAE 52536915.5.0000.0082). All the authors were consulted and consented to participate and submit to the Journal of Clinical Immunology. Consent to participate: Informed consent was obtained from all individual participants included in the study. The consent was read and explained for each participant, and all the text was previously revised by the ethical committee. Patients younger than 18 years old had a specific consent, and the parent or legal guardian also signed the consent to participate in the study. Consent for publication: The patient or in the case of patients less than 18 years old, his/her parent or legal guardian authorized the publication of the results without identifying their names. The patients were nominated by numbers to maintain their identity anonymous. Conflict of interest: No conflicts of interest related to this project. ASG belongs to the editorial board. ASG has been a consultant and/or speaker for CSL Behring, Takeda, and Catalyst. CMK has been a speaker for Takeda. PRJ has been a consultant and/or speaker for CSL Behring, Takeda, Novartis, and Sanofi. EM has been a speaker for Takeda, CSL Behring, Novartis, and Sanofi and a consultant for Takeda and CSL Behring. CSA has been a speaker for Takeda, CSL Behring, Sanofi, and Roche and a consultant for PTC, EUSA Pharma, and Sanofi., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Physical activity and sedentary behavior are treatable traits that may impact asthma control in distinct manners, but this impact remains poorly understood., Objective: To evaluate the influence of physical activity and sedentary behavior on clinical control in adults with moderate-to-severe asthma., Methods: This cross-sectional, multicentric study included 426 individuals with moderate-to-severe asthma. Assessments included physical activity and sedentary time (actigraphy), clinical asthma control (Asthma Control Questionnaire [ACQ]), quality of life (Asthma Quality of Life Questionnaire), anxiety and depression symptoms (Hospital Anxiety and Depression Scale), anthropometric data, and lung function. Participants were grouped according to physical activity levels and sedentary behavior., Results: Participants who walked ≥7500 steps/day presented better ACQ scores than those who walked <7500 steps/day (P < .05), independent of sedentary status. The percentage of patients with controlled asthma was higher in the active/sedentary (43.9%) and active/nonsedentary (43.8%) groups than in the inactive/sedentary (25.4%) and inactive/nonsedentary (23.9%) groups (P < .02). The likelihood of having uncontrolled asthma according to the treatable traits of physical inactivity (odds ratio [95% confidence interval]: 2.36 [1.55-3.59]), higher anxiety (2.26 [1.49-3.42]), and depression symptoms (1.95 [1.28-2.95]) was significant (P ≤ .002). Obesity and sedentary time were not associated with asthma control., Conclusions: Our results show that ≥7500 steps/day is associated with better asthma control independent of sedentary time in adults with moderate-to-severe asthma. Physical inactivity, anxiety, and depression symptoms are associated with higher odds of uncontrolled asthma. These results suggest that interventions should mainly focus on increasing physical activity rather than reducing sedentary time., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Previous research has suggested that most adults improve their asthma control after a short-term behavioral intervention program to increase physical activity in daily life (PADL). However, the characteristics of individuals who respond and do not respond to this intervention and the medium-term response remain unknown., Objective: This study aims to (1) identify the characteristics of adult responders and nonresponders with asthma to a behavioral intervention to increase physical activity and (2) evaluate the functional and clinical benefits in the medium term., Methods: This prospective pragmatic study will include adults with moderate to severe asthma who enroll in a behavioral intervention. All individuals will receive an educational program and an 8-week intervention to increase PADL (1 time/wk; up to 90 min/session). The educational program will be conducted in a class setting through group discussions and video presentations. Behavioral interventions will be based on the transtheoretical model using counseling, incentives, and individual feedback aiming to increase participation in physical activity. Motivational interviewing and guidelines for overcoming barriers will be used to stimulate individuals to reach their goals. Pre- and postintervention assessments will include the following: PADL (triaxial accelerometry), body composition (octopolar bioimpedance), barriers to PADL (questionnaire), clinical asthma control (Asthma Control Questionnaire), quality of life (Asthma Quality of Life Questionnaire), anxiety and depression levels (Hospital Anxiety and Depression Scale), and exacerbations. "Responders" to the intervention will be defined as those who demonstrate an increase in the number of daily steps (≥2500)., Results: In December 2021, the clinical trial registration was approved. Recruitment and data collection for the trial is ongoing, and the results of this study are likely to be published in late 2024., Conclusions: The intervention will likely promote different effects according to the clinical characteristics of the individuals, including asthma control, age, anxiety and depression levels, obesity, and several comorbidities. Identifying individuals who respond or do not respond to behavioral interventions to increase PADL will help clinicians prescribe specific interventions to adults with asthma., Trial Registration: ClinicalTrials.gov NCT05159076; https://clinicaltrials.gov/ct2/show/NCT05159076., International Registered Report Identifier (irrid): DERR1-10.2196/49032., (©Fabiano Francisco de Lima, Adriana Claudia Lunardi, David Halen Araújo Pinheiro, Regina Maria Carvalho-Pinto, Rafael Stelmach, Pedro Giavina‑Bianchi, Rosana Câmara Agondi, Celso RF Carvalho. Originally published in JMIR Research Protocols (https://www.researchprotocols.org), 31.08.2023.)

Objective: Environmental control includes measures to prevent exposure to common aeroallergens in an individual's home. Questionnaires are part of the clinical practice of health assessment, and are also widely used in research. Our aim was to develop and validate a questionnaire to identify possible sources of aeroallergens present in the indoor environment., Methods: This study describes the development, validation and application of a questionnaire. For content validation the Content Validation Index and Ordinal Cronbach's Alpha Index have been used; Polychoric Correlations for the agreement between judges; and an Exploratory Factor Analysis for the structure of the questionnaire, while for reliability assessment, Intraclass Correlation Coefficient has been applied., Results: Twenty-one doctors participated as judges to validate the questionnaire, which 204 patients answered. The Content Validity Index for all the questions on the "Clarity" aspect was 0.846 ± 0.152 and on the "Relevance" aspect, 0.954 ± 0.080. Cronbach's alpha coefficient for the "Clarity" aspect was 0.88 with a 95% confidence intervals (CI) and the "Relevance" aspect, 0.94 with a 95% CI. The average Intraclass Correlation Coefficient was 0.94 and all the F tests were highly significant., Conclusions: The questionnaire developed by our group was considered valid and reliable, and is capable of portraying the home environment without the need for a personal visit to the patient's home. This questionnaire would be a good tool to use in research or during patient consultations to assess the patient's home environment, as this latter assessment is essential for the management of patients with respiratory allergies.

Introduction: Systemic Mastocytosis comprises a group of neoplastic diseases characterized by clonal expansion and infiltration of mast cells into several organs. The diagnosis and treatment of this disease may be challenging for non-specialists., Objective: Make suggestions or recommendations in Systemic Mastocytosis based in a panel of Brazilian specialists., Method and Results: An online expert panel with 18 multidisciplinary specialists was convened to propose recommendations on the diagnosis and treatment of Systemic Mastocytosis in Brazil. Recommendations were based on discussions of topics and multiple-choice questions and were graded using the Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence Chart., Conclusion: Twenty-two recommendations or suggestions were proposed based on a literature review and graded according to the findings., Competing Interests: Conflicts of interest AMMC, AMS, AFS, FT, FLN, GFP, JLB, LLM, LFE, MGB and MVA declare no conflicts of interest. EDRPV has received research grants from Novartis, AbbVie and Onconova and presented scientific data in on behalf of Celgene and Novartis. FPSS has received research grants from Novartis, participated in advisory boards at Novartis and presented scientific data on behalf of Novartis, BMS, Amgen, AbbVie, Pfizer and Astellas. FSS has participated in advisory boards at Novartis. GAP has participated in advisory boards and presented scientific data on behalf of Novartis. KBBP has participated in advisory boards at Novartis, Astellas and GSK and presented scientific data on behalf of Novartis, Pintpharma, Wyeth, Janssen and EMS. PGB has participated in advisory boards at Novartis, Sanofi, AstraZeneca and Takeda. RCA has presented scientific data on behalf of Novartis., (Copyright © 2022 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U. All rights reserved.)

Background: SARS-CoV-2 enters lung cells via angiotensin-converting enzyme 2 (ACE2) receptor. Several studies suggest that interleukin-13, an important cytokine involved in T2 inflammation, reduces ACE2 expression, and therefore, asthma would not be a significant risk factor for the development of severe COVID-19. However, several asthma-related risk factors should be valued during the concurrent occurrence of asthma and COVID-19. The purpose of this study was to compare the evolution of asthma in patients who had COVID-19 with those who did not have the disease., Methods: This was an observational and retrospective study involving asthmatic patients followed up at a tertiary center. Patients were assessed for severity of asthma, atopy, comorbidities, and COVID-19. Worsening of asthma was considered when, during the period of Sept 2020 to Oct 2021, patients referred an increasing of asthma symptoms and a need to increment their maintenance therapy., Results: This study included 208 asthmatic patients, the mean age was 52.75 years, 79.81% were atopic asthmatics, and 59 (28.37%) had laboratory-confirmed coronavirus disease. Of all patients infected with the SARS-CoV-2, eleven (18.64%) needed hospitalization and required oxygen supply with an O2 mask. Comparing the worsening of asthma between patients who had COVID-19 and those who had not the disease, there was a statistically significant difference, 33.90 vs. 11.41%, respectively ( p < 0.001). There was no statistical significance regarding asthma comorbidities., Conclusion: This study assessed a group of asthmatic patients that had COVID-19, and that although the respiratory symptoms related to COVID-19 were mild to moderate, a subgroup of these asthmatic patients evolved with a chronic worsening of their asthma requiring an increment in asthma medication to control the disease., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Agondi, Menechino, Marinho, Kalil and Giavina-Bianchi.)

Objectives: The study describes a case series of hereditary angioedema with C1 Inhibitor Deficiency (C1INH-HAE) in order to corroborate six clinical warning signs "HAAAAE (H4AE)" to enable early identification of this disease., Methods: The authors analyzed the C1INH-HAE cohort to analyze the clinical aspects of the present study's patients and corroborate the six clinical warning signs of the Hereditary Angioedema Brazilian Guidelines. Data regarding demographics, the onset of disease, time to diagnosis, frequency of attacks per year, organs involved, triggers, crisis duration and their outcomes, and disease treatment were collected. Then the authors developed an acronym, H4AE, to help healthcare professionals remember the warning signs., Results: The authors included 98 patients in the study, with a mean age of 38.1 years, 67.3% being female, and 75.3% with a family history of HAE. HAE diagnosis was delayed, on average, 13.7 years after its initial manifestation. Exploratory laparotomy was reported by 26.9%, and orotracheal intubation by 21.3% of the present study's patients; 61.3% and 30.3% of them were admitted at least once in the hospital and in the intensive care unit, respectively. The authors constructed an acronym "H4AE" with the six warning signs of HAE: Hereditary, recurrent Angioedema, Abdominal pain, Absence of urticaria, Absence of response to antihistamines, Estrogen association., Conclusion: C1INH-HAE is still underdiagnosed and associated with high morbidity. The study showed clinical features of this disease, corroborating the warning signs, which may be useful in raising awareness and improving the diagnosis of C1INH-HAE. The authors suggest the acronym "H4AE" to remind the warning signs., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2022 HCFMUSP. Published by Elsevier España, S.L.U. All rights reserved.)