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Introduction: Good's Syndrome is a rare cause of immunodeficiency associated with thymoma. Patients with this syndrome are prone to infections with encapsulated microorganisms. The diagnosis may be delayed for a considerable time period even after the thymectomy. Case presentation: We describe the case of a 70-year-old woman with a background of thymectomy who presented with pneumonia and gram negative sepsis. Haemophilus influenzae was found in blood cultures. Moreover, there was evidence of impaired B and T cell immunity consistent with Good's Syndrome. She was commenced on immunoglobulin replacement following treatment of sepsis and remains well 18 months after the initial presentation. Conclusion: This case illustrates the importance of considering Good's Syndrome in the context of pneumonia and immunodeficiency associated with encapsulated organisms such as Haemophilus influenzae. This clinical entity is associated with a significant mortality and should be considered as a cause of immunodeficiency even years after thymectomy. Resumo: Introdução: A Síndrome de Good é uma causa rara da imunodeficiência associada ao timoma. Os pacientes com esta síndrome são propensos a infecções por microrganismos encapsulados. O diagnóstico pode ser atrasado por bastante tempo, mesmo após a timectomia. Apresentação do Caso: Descrevemos o caso de uma mulher de 70 anos com antecedentes de timectomia, que apresentava pneumonia e sepsis por agente gram negativo. O Haemophilus influenzae foi isolado em hemoculturas. Além disso, evidência de alterações da imunidade celular B e T, consistente com a Síndrome de Good. A doente iniciou terapêutica de substituição com imunoglobulina seguida de tratamento da sepsis e continua bem 18 meses após a apresentação inicial. Conclusão: Este caso ilustra a importância de considerar a Síndrome de Good no contexto da pneumonia e imunodeficiência associadas a organismos encapsulados, como Haemophilus influenzae. Esta entidade clínica está associada a uma mortalidade significativa e deve ser considerada como uma causa de imunodeficiência mesmo anos depois da timectomia. Keywords: Respiratory infections, Immunodeficiency, Thymoma, Palavras-chave: Infecções respiratórias, Imunodeficiência, Timoma

Abstract??The majority of patients with dermatitis herpetiformis (DH) have small intestinal enteropathy that mayresult in bone loss. The objective of this study was to evaluate bone mineral density (BMD) in DH and to examine whether dietary treatment or degree of the small intestinal lesion correlate with BMD. Twenty-five patients with DH (18 men) were investigated. Detailed dietary assessment and duodenal biopsies were performed on all patients before entry into the study. BMD at lumbar spine and femur was determined by DXA scan. Bone biomarkers, vitamin D, and parathyroid status were assessed. Twenty patients had enteropathy. None of the patients had hypovitaminosis D or secondary hyperparathyroidism. Resorption and formation markers were within normal limits. BMD Z-scores were not significantly different from expected (?0.38; CI, ?0.84 to 0.07) and femur (0.46; CI, ?0.06 to 0.97). There was no relationship between BMD Z-scores and the severity of the degree of enteropathy. We conclude that enteropathy of differing severity is present in 80% of patients with DH, but this is not associated with bone disease. [ABSTRACT FROM AUTHOR]

Celiac disease is caused by sensitivity to wheat gluten in genetically susceptible individuals. The etiological role of the other wheat-related cereals, barley, rye, and oats, is still debated. In order to investigate this issue further, in this study we examined the immune response of celiac mucosal T cell lines to fractions from all four cereals. Cell stimulation was assessed by measuring proliferation (employing 3H-thymidine incorporation) or cytokine (IL-2, IFN-γ) production. All five T cell lines demonstrated immunoreactivity to protein fractions from the four related cereals. In some cell lines, reactivity to wheat, barley, and rye was only evident when these cereal fractions had been pretreated with tissue transglutaminase. This study confirms the similar T cell antigenic reactivity of these four related cereals and has implications for their exclusion in the gluten-free diet. However, despite oats stimulation of T cell lines, this cereal does not activate a mucosal lesion in most celiac patients. [ABSTRACT FROM AUTHOR]

Abstract Data collection for the national registry for patients with primary immunodeficiency disorders in the Republic of Ireland commenced in 1996. One hundred and fifteen cases of primary immunodeficiency diseases were registered between December 1996 and February 2003. The most frequent primary immunodeficiency disorders were antibody deficiency (n = 53) and complement deficiency (n = 32). In addition, patients with T cell deficiency (n = 11) and chronic granulomatous disease (n = 11) were identified. A small number of patients with Wiskott–Aldrich syndrome, natural killer cell deficiency, DiGeorge syndrome and chronic mucocutaneous candidiasis were also registered. Comparison of our data with that recently reported in the European registry revealed that complement deficiency was more prevalent in the Republic of Ireland compared to other European countries. Results of our registry point to a significant prevalence of primary immunodeficiency disorders in the Republic of Ireland (2.9 cases per 100.000 population). However, it is likely that these figures underestimate the true prevalence of such cases in the country. We hope, with increased awareness of the national registry among primary care physicians, that more patients will be included and we will be able to identify accurately the frequency and the distribution of these disorders. [ABSTRACT FROM AUTHOR]

Idiopathic anaphylaxis (IA) is a severe allergic reaction without identifiable external triggers, presenting significant challenges in diagnosis and management. However, growing evidence suggests that many cases classified as idiopathic may actually be driven by cofactors such as exercise, hormonal fluctuations, medications, or hidden allergens. This mini-review explores the evolving understanding of IA, highlighting the role of these cofactors in triggering or amplifying anaphylactic reactions. It emphasizes how advances in diagnostic tools, including component-resolved diagnostics, are helping to identify previously undetected allergens, leading to more accurate diagnoses and reducing the prevalence of true idiopathic cases. As our knowledge of anaphylaxis and its underlying mechanisms deepens, the need for comprehensive evaluations that account for cofactor involvement becomes increasingly clear. Continued research in this area is essential to improve patient outcomes and better manage this complex condition., 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., (© 2024 Elkhalifa, Elbashir and Abuzakouk.)

Competing Interests: The authors report no conflicts of interest in this communication.

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic vasculitis characterized by eosinophil-rich, necrotizing granulomatous inflammation primarily affecting the respiratory tract with necrotizing vasculitis of small- to medium-sized arteries. In this case series, we retrospectively evaluated the efficacy and safety of mepolizumab in seven patients diagnosed with EGPA who presented to the Department of Allergy and Clinical Immunology at Cleveland Clinic Abu Dhabi. The variables assessed before and after mepolizumab treatment included Birmingham Vasculitis Activity Score (BVAS), prednisolone dose, Asthma Control Test (ACT) score, and blood eosinophil count (BEC). We found a significant reduction in BVAS and prednisolone dosage with clinical improvements in asthma symptoms after treatment with mepolizumab. Our case series, the first from the Middle East on the use of mepolizumab in EGPA, demonstrates that mepolizumab is a safe and effective treatment for patients with EGPA., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Abuzakouk et al.)

Background: Angioedema (AE) manifests with intermittent, localized, self-limiting swelling of the subcutaneous and/or submucosal tissue. AE is heterogeneous, can be hereditary or acquired, may occur only once or be recurrent, may exhibit wheals or not, and may be due to mast cell mediators, bradykinin, or other mechanisms. Several different taxonomic systems are currently used, making it difficult to compare the results of studies, develop multicenter collaboration, and harmonize AE treatment., Objective: We developed a consensus on the definition, acronyms, nomenclature, and classification of AE (DANCE)., Methods: The initiative involved 91 experts from 35 countries and was endorsed by 53 scientific and medical societies, and patient organizations. A consensus was reached by online discussion and voting using the Delphi process over a period of 16 months (June 2021 to November 2022)., Results: The DANCE initiative resulted in an international consensus on the definition, classification, and terminology of AE. The new consensus classification features 5 types and endotypes of AE and a harmonized vocabulary of abbreviations/acronyms., Conclusion: The DANCE classification complements current clinical guidelines and expert consensus recommendations on the diagnostic assessment and treatment of AE. DANCE does not replace current clinical guidelines, and expert consensus algorithms and should not be misconstrued in a way that affects reimbursement of medicines prescribed by physicians using sound clinical judgment. We anticipate that this new AE taxonomy and nomenclature will harmonize and facilitate AE research and clinical studies, thereby improving patient care., (Copyright © 2024. Published by Elsevier Inc.)

Allergic rhinitis (AR) is a chronic inflammatory condition of the upper airways caused by a type I hypersensitivity reaction triggered by environmental allergens. AR is associated with significant morbidity and affects patients' quality of life, emotional well-being, productivity, and cognitive functioning. As AR prevalence and morbidity have increased significantly worldwide, similar observations have been noted in the United Arab Emirates (UAE) with AR becoming a potential public health issue. Management of AR in the UAE is mainly provided by non-allergy specialists relying on first-line treatments such as intranasal steroids and antihistamines, with often suboptimal and short-term efficacy. Allergen Immunotherapy (AIT) is the only currently available disease-modifying treatment option in the form of either subcutaneous or sublingual allergen immunotherapy that has been proven to have long-term benefits. This article aims to provide recommendations regarding the use of AIT for managing AR in the UAE, considering both the current landscape in the Emirati healthcare system and local experience., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have received an honorarium from Biologix FZCO for the time invested in generating this manuscript, including making substantial contributions to data interpretation, reviewing the article critically for its scientific content, and revising and approving the final version independently. Financial relationships: Eike Wuestenberg declare(s) employment and stock/stock options from ALK. Tushar Arora declare(s) employment from Biologix FZCO. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Zaitoun et al.)

Objective: The United Arab Emirates (UAE), with its characteristic local population, geography, and history, presents several risk factors for cardiovascular diseases (CVDs) in obese individuals. Obesity and its associated complications, including diabetes, atherogenic dyslipidemia, and CVDs leading to significant health risks. In the present study, "Youths" defined as young people between 18 and 22 years. We assessed dyslipidemia, inflammation, and oxidative stress biomarker levels and their association with endothelial dysfunction (ED) in both overweight/obese and normal weight youths of UAE., Methods: There were 160 youths with overweight/obese (BMI ≥ 25 kg/m 2 ) patients and healthy age- and sex-matched normal weight (BMI ≤ 24.9 kg/m 2 ) as controls participated in this study. The anthropometric data and blood samples were collected to assess the biomarkers for dyslipidemia, inflammation, oxidative stress, ED from all the youths., Results: The overall mean age and male-to-female ratio were 20±1.5years and 1.0:1.2, respectively. There was statistically significant difference in HDL-C (p<0.001), triglycerides (TG) (p<0.001), ApoA (p=0.002), ApoB/ApoA ratio (p=0.009) between the overweight/obese and normal weight youths. Among, inflammatory markers: hs-CRP, IL-6, TNF-α also showed significant p<0.001 and oxidative stress markers: DNA/RNA Damage, catalase and nitric oxide (NO) showed significant p<0.001 between groups. Spearman correlation of ED markers with lipid profile markers showed Vitamin C levels positively correlated with HDL-C (p<0.001) and negatively correlated with glucose (p<0.001). ICAM-1showed significant negative correlation with HDL-C (p<0.01) and ApoA (p<0.001) but positive correlation with TG (p<0.01) and HbA1c (p<0.001) among groups. Spearman correlation of ED markers with inflammatory/oxidative stress biomarkers showed Vitamin C levels negatively correlated with ferritin (p < 0.001), NO (p < 0.001), GGT (p < 0.001), and ALT (p < 0.001) levels. The ICAM-1showed significant positive correlation with hs-CRP (p < 0.01), IL-6 (p < 0.001), TNF-α (p < 0.01), GGT (p < 0.05), and ALT (p < 0.05) in both groups., Conclusion: This study revealed a strong link between the biomarkers of dyslipidemia, inflammation, and oxidative stress with ED in overweight/obese patients. This study might be used to predict future cardiovascular events in this population., Competing Interests: The authors report no conflicts of interest in this work., (© 2024 Sharma et al.)

This report describes a 48-year-old man who presented with a month history of weakness and paraesthesia associated with severe pain of all four limbs. Initially diagnosed and treated as Guillain Barre syndrome due to the severity of his extremity weakness, it was later discovered to be eosinophilic granulomatous polyangiitis (EGPA). Mononeuritis multiplex should not be underestimated or overlooked in the setting of diagnosing EGPA and requires prompt treatment with biologics to limit the permanent consequences on patient's quality of life with regard to developing limb weakness and pain.  Although peripheral neuropathy, namely, mononeuritis multiplex, is not the most common feature of EGPA, it is important to consider it in order not to delay treatment with biologic agents that as seen in our patient can both halt the progress of the disease as well as give the patient a better quality of life., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Al Saadi et al.)

Eosinophilic esophagitis (EoE) is a chronic, progressive, type 2 inflammatory esophageal disease presenting as dysphagia to solid food and non-obstructive food impaction. Knowledge gaps exist in its diagnosis and management. These expert recommendations focused on the diagnosis of EoE in the United Arab Emirates. An electronic search of PubMed and Embase databases was used to gather evidence from systematic reviews, randomized controlled trials, consensus papers, and expert opinions from the last five years on the diagnosis of EoE. The evidence was graded using the Oxford system. Literature search findings were shared with the expert panel. A 5-point scale (strongly agree, agree, neither agree nor disagree, disagree, and strongly disagree) was used, and a concordance rate of >75% among experts indicated agreement. Using a modified Delphi technique, 18 qualified experts provided 17 recommendations. Eleven statements achieved high agreement, four got moderate agreement, and two got low agreement. Challenges exist in diagnosing EoE, particularly in children. Esophageal biopsies were crucial in diagnosis, irrespective of visible mucosal changes. Further research on diagnostic tools like endoscopic mucosal impedance and biomarkers is needed. Diagnosis relies on esophageal biopsies and symptom-histology correlation; however, tools like EoE assessment questionnaires and endoscopic mucosal impedance could enhance the accuracy and efficiency of EoE diagnosis. The diagnosis of EoE is challenging since the symptoms seldom correlate with the histological findings. Currently, diagnosis is based on patient symptoms and endoscopic and histological findings. Further research into mucosal impedance tests and the role of biomarkers is needed to facilitate diagnosis., Competing Interests: The authors have declared financial relationships, which are detailed in the next section., (Copyright © 2024, Awadhi et al.)

Background: Data on eosinophilic gastrointestinal disorders (EGIDs) are limited in the United Arab Emirates., Objective: This study aimed to describe the distribution of eosinophilic gastritis/gastroenteritis (EoG/EGE) and eosinophilic esophagitis (EoE) among patients whose data are recorded on the Cleveland Clinic Abu Dhabi (CCAD) systems., Methods: Anonymized aggregate data were obtained from the Clinerion Patient Network Explorer (PNEx ® ) network installed at the CCAD on patients diagnosed with EoG/EGE (n=17, of whom 11 (64.7%) were females) or with EoE (n=118, of whom 35 (29.7%) were females) between 2015 and 2022; 63 (53.4%) were diagnosed between 2020 and 2022., Results: The vast majority of EoE patients visited the gastroenterology clinic (111, 94.1%), followed by the primary care (70, 59.3%), the emergency medicine (60, 50.8%), and the immunology (59, 50%) units. A total of 84 patients with EoE also presented with another type-2 inflammatory condition including allergic rhinitis, food allergy, and atopic dermatitis. Proton pump inhibitors and corticosteroids were widely prescribed, and four patients with EoE have been commenced on biologics since 2020. However, heterogeneity of medication use was identified, warranting local clinical practice guidelines., Conclusion: A referral network could be established with local and regional reach, which relies on a multidisciplinary approach for the management of EoE and associated type 2 co-morbidities. More studies are needed to better depict the clinical picture of patients with EGIDs in general and EoE, in particular, in terms of diagnosis, pharmacological treatment, non-medical interventions, disease course, and patient-reported outcomes., Competing Interests: The authors have declared financial relationships, which are detailed in the next section., (Copyright © 2024, Abuzakouk et al.)

Chronic rhinosinusitis with nasal polyps (CRSwNP) is a chronic and often debilitating inflammatory condition of the nasal and paranasal tissues. An expert panel of specialists from the Gulf region (the Kingdom of Saudi Arabia, Kuwait, Oman and the United Arab Emirates) and from Egypt gathered to evaluate existing guidance and develop regional guidance on the management of CRSwNP through a consensus approach. The present article presents the main observations and recommendations from this panel. CRSwNP diagnosis requires the presence of bilateral, endoscopically visualized polyps in the middle meatus (via nasal endoscopy or CT). In most patients, CRSwNP is mediated through predominantly type 2 inflammatory processes and is often observed in patients with asthma and other allergic disease. While many patients respond to medical treatment (principally topical irrigation and intranasal corticosteroids, and adjunctive short-term use of systemic corticosteroids), clinical management of CRSwNP is challenging, and a multidisciplinary approach for complete evaluation and treatment is recommended. Patients with more severe/uncontrolled disease (despite adequate medical therapies) require a complete endoscopic sinus surgery (ESS), although outcomes can be unsatisfactory, and further revision surgery is common. Biological therapies targeting underlying inflammatory processes offer additional, effective treatment options for those patients with persistent symptoms despite complete ESS, and also in those patients where surgery may be contraindicated., Competing Interests: RA-A has received honorarium from Sanofi; SA received lecture and advisory board honoraria from Sanofi and GSK; MA-A received lecture and advisory board honoraria from Sanofi, AstraZeneca, and GSK. The authors report no other conflicts of interest in this work., (© 2023 Marglani et al.)

Background: Bronchiectasis is a widely prevalent airway disease characterized by airway dilatation and recurrent infections, that can lead to respiratory failure in severe cases. The etiology of bronchiectasis varies geographically, but there is a lack of published data examining its etiology specifically within the Middle Eastern population., Methods: We conducted a retrospective analysis of our bronchiectasis patient registry, extracting clinical and demographic characteristics from electronic medical records. Quantitative variables were presented as the median and interquartile range (IQR), while categorical variables were expressed as numbers and percentages. Statistical comparisons for continuous characteristics were performed using the t-test, and significance was determined by a p-value less than 0.05., Results: In total we analysed 260 records (63% female, 37% male), with median age of 58 years (interquartile range (IQR) 38-71), Body Mass Index (BMI) 25.8(IQR 22-30), forced expiratory volume in the first second (FEV1) %predicted 65 (IQR 43-79) and FEV1/forced vital capacity (FVC) 0.76 (0.67-0.86). Sixty-five cases (25%) were post-infectious in aetiology (excluding post-TB - n:27 10.4%). Forty-eight (18.5%) patients were labelled idiopathic, while Primary Ciliary Dyskinesia (PCD) accounted for 23 (8.8%) cases. Pseudomonas aeruginosa was the most common colonizing organism (32.7%), followed by Haemophilus influenzae (9.2%) and Methicillin-Sensitive Staphylococcus aureus(6.9%). At the time of review, 11 patients had died (median age, FEV %predicted, and bronchiectasis severity index (BSI) 59 years, 38% and 15.5 respectively), all due to respiratory failure, and as expected, all were classed severe on BSI. The BSI score was available for 109 patients, of which 31(28%) were classed mild, 29(27%) were moderate, and 49 (45%) were classed severe. The median BSI score was 8 (IQR 4-11). On dividing the patients according to obstructive vs. restrictive spirometry, we found that patients with FEV1/FVC < 0.70 had significantly higher BSI (10.1 vs. 6.9, p-value < 0.001) and that 8 out of the 11 deceased patients had FEV1/FVC < 70%., Conclusions: In our study, post-infectious, idiopathic, and PCD were identified as the most common etiologies of bronchiectasis. Additionally, patients with obstructive spirometry appeared to have a worse prognosis compared to those with restrictive spirometry., (© 2023. The Author(s).)

Background: Hereditary angioedema (HAE), a potentially life-threatening genetic disorder due to C1 inhibitor deficiency in most cases, is characterized by sudden and/or recurrent attacks of angioedema (subcutaneous/submucosal swellings). The global World Allergy Organization (WAO)/European Academy of Allergy and Clinical Immunology (EAACI) International guideline for HAE management is comprehensive, but the implementation of this guideline may require regional adaptation considering the diversity in disease awareness, type of medical care systems, and access to diagnostics and treatment. The aim of this Delphi initiative was to build on the global guideline and provide regional adaptation to address the concerns and specific needs in the Middle East., Methods: The Consensus panel comprised 13 experts from the Middle East (3 from the United Arab Emirates, 3 from Saudi Arabia, 2 from Lebanon, 2 from Kuwait, 2 from Oman and 1 from Qatar) who have more than 2 decades of experience in allergy and immunology and are actively involved in managing HAE patients. The process that was carried out to reach the consensus recommendation included: 1.) A systematic literature review for articles related to HAE management using Ovid MEDLINE. 2.) The development of a questionnaire by an internationally acclaimed expert, with 10 questions specific to HAE management in the Middle East. 3.) Experts received the questionnaire via email individually and their answers were recorded (email/interview). 4.) A virtual consensus meeting was organized to discuss the questionnaire, make amends if needed, vote, and achieve consensus., Results: The questionnaire comprised 10 questions, each with 2 or more statements/recommendations on which the regional experts voted. A consensus was reached based on a 70% agreement between the participants. The key highlights include: 1) HAE experts in the Middle East emphasized the importance of a positive family history for arriving at a diagnosis of HAE. 2) The number of episodes per month or per 6-month period and severity should be used, together with other markers, to determine the need for prophylaxis. 3) Disease status should be monitored by periodic visits and the use of patient-reported outcome measures such as the angioedema activity score and the angioedema control test. 4) Attenuated androgens and tranexamic acid may be considered for long-term prophylaxis, if lanadelumab, C1-Inhibitor or berotralstat are not available., Conclusion: This consensus recommendation may help to educate healthcare practitioners in the Middle East and unify their approach to the diagnosis and management of HAE., (© 2022 The Authors.)

Normocomplementemic urticarial vasculitis is a rare autoimmune disorder characterized by leukocytoclasia, fibrin deposits, and extravasated erythrocytes affecting multiple organ systems. Current treatment modalities, including corticosteroids and immunosuppressive agents, are of limited efficacy and an expansive side effect profile. Omalizumab has been reported to be effective in urticarial vasculitis, but its long-term effectiveness and tolerability have not yet been evaluated. We report a case of long-standing normocomplementemic urticarial vasculitis treated with omalizumab only, for almost 3 years. The patient reported a significant improvement in quality of life after the first few doses with a significant improvement in the urticaria control test. The treatment was well tolerated and no adverse events were reported after 3 years. Our patient was treated with 300 mg of omalizumab, as it was previously linked with a better improvement in quality of life. We were able to extend our patient&rsquo;s treatment intervals, suggesting that this is feasible in patients treated with omalizumab who achieve a complete response. We recommend that larger and long-term studies are conducted to assess the efficacy and effectiveness of omalizumab in patients with urticarial vasculitis. J Drugs Dermatol. 2022;21(10):1124-1126. doi:10.36849/JDD.6739.

Aim: To explore and compare the efficacy of standard (300 mg every 2 weeks) and extended (300 mg every 4 weeks) dosing regimens of lanadelumab for long-term prophylaxis of hereditary angioedema (HAE)., Methods: We conducted a retrospective chart review of all patients with HAE on lanadelumab, which identified a total of 9 patients: 5 females and 4 males. The median age of patients was 31 years (IQR 20.7). The mean number of attacks per month before starting lanadelumab was 5.9 (SD 6.3). Patients were started on 300 mg of lanadelumab subcutaneously, every 2 weeks (standard group, n = 5) or every 4 weeks (extended group, n = 4)., Results: We observed a statistically significant improvement in the number of angioedema attacks per month in all 9 patients ( p  = 0.007). Five out of 9 patients (56%) achieved complete remission from attacks after starting lanadelumab. The effect of lanadelumab on number of angioedema attacks was significant in both groups; extended group ( p  = 0.03) and standard group ( p  = 0.01)., Conclusion: Lanadelumab is a safe and effective agent for long-term prophylaxis of HAE. An extended dosing regimen was equally effective as prophylaxis compared to a standard regimen. Further studies are needed to compare the 2 regimens in a larger patient group., (© 2022 The Author(s).)

Background: While crucial to the assessment and improvement of asthma control, insights on treatment practices in patients with severe diseases across Gulf nations are lacking. This observational study describes the treatment patterns of adolescents and adults with severe asthma across four countries of the Gulf region and evaluates current levels of asthma control; quality of life (QoL); exacerbation frequency; and the application of cellular, protein, and respiratory biomarkers in assessing asthma severity and inflammation., Methods: Patients (aged >12 years, body weight ≥40 kg) with clinician-diagnosed, severe asthma (guided by the 2018 Global Initiative for Asthma definition) were included in this cross-sectional, multicenter, observational study conducted in the four Gulf countries of Kuwait, Oman, Qatar, and the United Arab Emirates. Data on demographics, treatment patterns, and laboratory parameters (blood eosinophil count [BEC], levels of serum immunoglobulin E [IgE], and fractional exhaled nitric oxide [FeNO]) were extracted from the medical records of patients during a 12-month retrospective period and transcribed onto case report forms. At the Enrollment visit, patients assessed their asthma control and QoL with the self-administered Asthma Control Questionnaire (ACQ) and a standardized version of the Asthma Quality of Life Questionnaire (AQLQ(S)), respectively., Results: Among the 243 patients analyzed, (mean [standard deviation (SD)] age, 48.4 [13.9] years; female, 67.5%), the inhaled corticosteroid (ICS)/long-acting β 2 agonist (LABA) combination was the most prescribed asthma medication (n = 240; 98.8%). Most patients were classified as "uncontrolled," (n = 173; 71.2%) and the majority (n = 206; 84.8%) experienced ≥1 exacerbation(s) in the preceding 12 months. The mean (SD) ACQ score was 2.1 (1.2), which indicated uncontrolled asthma, and the mean (SD) total AQLQ(S) score was 4.7 (1.4), suggesting "some limitation" in overall QoL. BECs during the 12-month period were elevated in most patients (>300 cells/μL [n = 183; 41.7%], 150-300 cells/μL [n = 138; 31.4%], <150 cells/μL [n = 118; 26.9%]), suggesting an eosinophilic asthma phenotype, although no standardized threshold by which to define eosinophilia has yet been confirmed. This study revealed that the biomarkers BEC, serum IgE, and FeNO concentrations were obtained inconsistently by the participating centers., Conclusions: Despite recommended ICS/LABA therapy being prescribed to most patients for their severe disease, the majority experienced uncontrolled asthma and exhibited elevated BECs. These findings indicate the need for enhanced treatment strategies to improve and sustain asthma control in the Gulf region., (© 2022 The Author(s).)

This update and revision of the international guideline for urticaria was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA²LEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), the European Dermatology Forum (EDF; EuroGuiDerm), and the Asia Pacific Association of Allergy, Asthma and Clinical Immunology with the participation of 64 delegates of 50 national and international societies and from 31 countries. The consensus conference was held on 3 December 2020. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease that presents with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous or inducible urticaria is disabling, impairs quality of life, and affects performance at work and school. This updated version of the international guideline for urticaria covers the definition and classification of urticaria and outlines expert-guided and evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria., (© 2021 GA²LEN. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Chronic rhinosinusitis (CRS) is defined as the inflammation of nose and paranasal sinuses, affecting the patients' quality of life and productivity. Chronic rhinosinusitis with nasal polyps (CRSwNP) is a principal clinical entity confirmed by the existence of chronic sinonasal inflammation and is characterized by anterior or posterior rhinorrhea, nasal congestion, hyposmia and/or facial pressure or facial pain. Several epidemiologic studies have revealed wide variations in the incidence of CRS among regions globally ranging from 4.6% to 12%. The Gulf countries are also witnessing an unprecedented burden of CRSwNP. According to the current clinical guidelines, glucocorticosteroids and antibiotics are the principal pharmacotherapeutic approaches. Endoscopic sinus surgery is recommended for those who have failed maximal pharmacotherapy. Recently, biologics are considered as an alternative best approach due to the complications associated with medical therapy and surgery. However, precise data on the clinical position of biologic agents in the management of CRSwNP in the Gulf region is not available. The present review article addresses the current diagnostic and management approaches for CRSwNP and also emphasizes the role of emerging biologics in the current treatment strategies for CRSwNP in the Gulf region. Further, a consensus protocol was convened to rationalize the guideline recommendations, strategize the best practices with biologics, and develop clinical practice guidelines for all primary-care specialists in the Gulf region. The consensus-based report will be a useful reference tool for primary-care physicians in primary-healthcare settings, regarding the appropriate time for the initiation of biological treatment in the Gulf region., Competing Interests: Sanofi Genzyme has provided advisory board honoraria for authors who participated in Sanofi Genzyme advisory board, but they were not paid for writing the publication. The authors report no other potential conflicts of interest for this work., (© 2022 Al-Ahmad et al.)

Objective: To clarify whether in adults with a nonobstructive spirometry a reduced FEF25-75% is associated with a positive methacholine challenge test (MCT)., Methods: Data was collected for all the patients who had a MCT done between April 2014 and January 2020 but had nonobstructive baseline spirometry. Logistic regression was utilized to estimate the log odds of a positive methacholine test as a function of FEF25-75% and also for age, gender, BMI, FEV1, and FEV1/FVC., Results: Out of 496 patients, 187 (38%) had a positive MCT. Baseline characteristics in two groups were similar except that patients with positive MCT were younger (32 ± 11.57 vs. 38 ± 13.25 years, respectively, p < 0.001). Mean FEF25-75% was lower in MCT positive (3.12 ± 0.99 L/s) vs. MCT negative (3.39 ± 0.97 L/s) patients, p = 0.003. Logistic regression results suggest that MCT outcome is inversely related to FEF25-75%, age, and gender. Specifically, as FEF25-75% percentage of predicted value increases, the log odds of a positive MCT decrease (odds ratio (OR) = 0.90, 95% confidence intervals (CI) = 0.84-0.96, p = 0.002). Also, as age increases, the log odds of a positive MCT decrease (OR = 0.95, 95%CI = 0.94-0.97, p < 0.001)., Conclusions: Reduced FEF25-75% in adults with nonobstructive spirometry can predict a positive response to MCT in younger patients. However, this relationship becomes weaker with increasing age., Competing Interests: No conflict of interest has been declared by the authors., (Copyright © 2021 Irfan Shafiq et al.)

Context.—: In the face of the coronavirus disease 2019 (COVID-19) pandemic response, it was worthwhile to test the safety and efficacy of COVID-19 convalescent plasma (CCP) transfusion., Objective.—: To establish a CCP donation program based on the availability of recovered COVID-19 patients and the practical limitations in recruiting clinically valid donors in a multicultural setting., Design.—: From March to June 2020, we developed a program for collection of COVID-19 CCP as part of the treatment options for patients affected with COVID-19. From an initial population of 3746 candidates, only those with positive polymerase chain reaction results in at least 2 separate tests were considered. This filter reduced the eligible donor pool to 488 patients. After other exclusions were applied, such as language barrier, age, accessibility to donation, and comorbidities, the final count was 267 potentially eligible donors, which represented only 54.7% (267 of 488) of preselected candidates., Results.—: Eighty donors were called. Approximately a third of the calls provided additional challenges as outlined by the following 4 reasons: limited functional understanding of English; schedule availability due to rotating work timetables; transportation restrictions since public transport services were severely restricted during lockdown; and lost to follow-up. Finally, a total of 38 valid donors participated, upon whom 45 apheresis procedures were performed., Conclusions.—: As a summary of our experience, we can conclude that despite the limitations we were able to establish an effective program. A total of 90 units of CCP were collected before the pandemic curve began to flatten toward the end of June 2020.

Background: Information/communication technologies such as mobile phone applications (apps) would enable chronic urticaria (CU) patients to self-evaluate their disease activity and control. Yet, recently Antó et al (2021) reported a global paucity of such apps for patients with CU. In this analysis, we assessed patient interest in using apps to monitor CU disease activity and control using questions from the chronic urticaria information and communication technologies (CURICT) study., Methods: The methodology for CURICT has been reported. Briefly, a 23-item questionnaire was completed by 1841 CU patients from 17 UCAREs across 17 countries. Here, we analyzed patient responses to the CURICT questions on the use of apps for urticaria-related purposes., Results: As previously published, the majority of respondents had chronic spontaneous urticaria (CSU; 63%; 18% chronic inducible urticaria (CIndU) [CIndu]; 19% with both), were female (70%) and in urban areas (75%). Over half of patients were very/extremely interested in an app to monitor disease activity (51%) and control (53%), while only ∼1/10 were not. Patients with both urticaria types versus those with CSU only (odds ratio [OR], 1.36 [1.03-1.79]) and females versus males (OR [95% CI], 1.47 [1.17-1.85]) were more likely to be very to extremely interested in an app to assess disease control., Conclusions: Overall, half of the patients with CU were very to extremely interested in using an app to assess their disease activity and control. Development of well-designed apps, specific to disease types (CSU, CIndU, CSU + CIndU, etc), validated by experts across platforms would help improve the management and possibly outcomes of CU treatment while providing important patient information to be used in future research., (© 2021 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Objective: Cystic fibrosis (CF) is the commonest life-limiting inherited illness in the Caucasian population but is uncommon in the Middle East, and so the genotypes and clinical course of disease in this population is not well known., Material and Methods: In this retrospective observational study, we collected and reviewed the data on CF mutations, body mass index (BMI), lung function, microbiology, and the demographics in adult CF patients in the United Arab Emirates (UAE)., Results: Data was reviewed for 39 adult CF patients. The median age of adult CF patients presenting to our clinic was 25 years (interquartile range (IQR) 22-31), the median BMI was 19 (IQR 17-22), and the median percentage predicted forced expiratory volume at 1 second (FEV1) was 49.5% (IQR 38.5-62.5). S549R was the commonest mutation (n = 11, 28%) followed by ∆F508 (n = 9, 23%). Only 5 (13%) out of 39 patients were heterozygote for CF mutations which reflects the high level of consanguinity in the region. Twelve (30%) patients were diagnosed after the age of 16, and in total, 19 (48%) were diagnosed after the age of 10. Thirty-two (82%) of patients are pseudomonas colonized, and 31% had 3 or more exacerbations in the last 12 months., Conclusion: The CF mutation patterns in the UAE are different from western populations with low ∆F508 prevalence, with the presence of rare mutations more specific to this region and a high rate of homozygosity. Late diagnosis, high pseudomonas colonization rate, and exacerbation frequency remain a problem in this region and lead to poor long-term outcomes.

Background: Patients with chronic urticaria (CU) are increasingly using information and communication technologies (ICTs) to manage their health. What CU patients expect from ICTs and which ICTs they prefer remains unknown. We assessed why CU patients use ICTs, which ones they prefer, and what drives their expectations and choices., Methods: In this cross-sectional study, 1841 patients across 17 countries were recruited at UCAREs (Urticaria Centers of Reference and Excellence). Patients with CU who were >12 years old completed a 23-item questionnaire., Results: Most patients were interested in receiving disease information (87.3%), asking physicians about CU (84.1%), and communicating with other patients through ICTs (65.6%). For receiving disease information, patients preferred one-to-one and one-to-many ICTs, especially web browsers. One-to-one ICTs were also the ICTs of choice for asking physicians about urticaria and for communicating with other patients, and e-mail and WhatsApp were the preferred ICTs, respectively. Many-to-many ICTs such as Facebook, Instagram, LinkedIn, and Twitter were least preferred for all 3 purposes. Living in rural areas and higher education were linked to higher odds of being interested in receiving disease information, asking physicians, and communicating with patients through ICTs., Conclusions: Most patients and especially patients with higher education who live in rural areas are interested in using ICTs for their healthcare, but prefer different ICTs for different purposes, ie, web browsers for obtaining information, e-mail for asking physicians, and WhatsApp for communicating with other patients. Our findings may help to improve ICTs for CU., Competing Interests: I Cherrez-Ojeda has no conflicts of interest. E Vanegas has no conflicts of interest. A Cherrez has no conflicts of interest. M Felix has no conflicts of interest. K Weller is or recently was a speaker and/or advisor for, and/or has received research funding from: Biocryst, CSL Behring, Dr. Pfleger, FAES, Moxie, Novartis, Shire/Takeda, and Uriach. M Magerl is or recently was a speaker and/or advisor for, and/or has received research funding from Biocryst, CSL Behring, Kalvista Pharmaceuticals, Moxie, Novartis, Pharming, and Shire/Takeda. RR Maurer has no conflicts of interest. VL Mata has no conflicts of interest. A Kasperska-Zajac has no conflicts of interest. A Sikora has no conflicts of interest. D Fomina is or recently was a speaker and/or advisor for, and/or has received research funding from: AstraZeneca, CSL Behring, Glaxo SmithKline, MSD, Novartis, Sanofi, and Shire/Takeda. E Kovalkova has no conflicts of interest. K Godse has no conflicts of interest. N Dheeraj Rao has no conflicts of interest. M Khoshkhui has no conflicts of interest. S Rastgoo has no conflicts of interest. RFJ Criado has no conflicts of interest. M Abuzakouk has no conflicts of interest. D Grandon has no conflicts of interest. MBA van Doorn is or recently was a speaker and/or advisor for, and/or has received research funding from Abbvie, BMS, Celgene, Janssen Cilag, LEO Pharma, Lilly, MSD, Novartis, Pfizer, and Sanofi-Genzyme. S Valle has no conflicts of interest. E Magalhães de Souza Lima has no conflicts of interest. SF Thomsen is or recently was a speaker and/or advisor for, and/or has received research funding from: Abbvie, AstraZeneca, Celgene, Eli Lilly, Janssen, LEO Pharma, Novartis, Pierre Fabre, Roche, Sanofi, and UCB. GD Ramón has no conflicts of interest. EE Matos Benavides has no conflicts of interest. A Bauer is or recently was a speaker and/or advisor for, and/or has received research funding from Novartis, Sanofi, Genentech, Leo Pharma and Shire/Takeda. Ana M Giménez-Arnau has held roles as a Medical Advisor for Sanofi, Uriach, Genentech, Novartis, Amgen , ThemoFisher, Roche, Almirall, and has research grants supported by Instituto Carlos III- FEDER, Novartis, and Uriach; she also participates in educational activities for Almirall, Genentech, Glaxo SmithKline, LEO Pharma, Menarini, MSD, Novartis, Sanofi, Avene and Uriach. E Kocatürk is or recently was a speaker and/or advisor for Bayer, Novartis, and Sanofi. C Guillet has no conflicts of interest. JI Larco is or recently was a speaker and/or advisor for: FAES, Novartis, and Sanofi. Z-T Zhao has no conflicts of interest. M Makris is or recently was a speaker and/or advisor for, and/or has received research funding from AstraZeneca, Chiesi, Glaxo SmithKline, Novartis, and Sanofi. C Ritchie has no conflicts of interest. P Xepapadak reports personal fees from Galenica Greece, Glaxo SmithKline, Nestle, Novartis, Nutricia, and Uriach, outside the submitted work. LF Ensina is or recently was a speaker and/or advisor for, and/or has received research funding from Novartis, Sanofi, and Takeda. S Cherrez has no conflicts of interest. M Maurer is or recently was a speaker and/or advisor for, and/or has received research funding from: Allakos, Alnylam, Aralez, AstraZeneca, Biocryst, Blueprint, CSL Behring, FAES, Genentech, Kalvista Pharmaceuticals, LEO Pharma, Menarini, Moxie, MSD, Novartis, Pharming, Pharvaris, Roche, Sanofi, Shire/Takeda, UCB, and Uriach., (© 2021 The Author(s).)

(1) Background: There are limited data regarding the efficacy of convalescent plasma (CP) in critically ill patients admitted to the intensive care unit (ICU) due to coronavirus disease 2019 (COVID-19). We aimed to determine whether CP is associated with better clinical outcome among these patients. (2) Methods:&nbsp;A&nbsp;retrospective single-center study including adult patients with laboratory-confirmed SARS-CoV-2 infection admitted to the ICU for acute respiratory failure. The primary outcome was time to clinical improvement, within 28 days, defined as patient discharged alive or reduction of 2 points on a 6-point disease severity scale. (3)&nbsp;Results:&nbsp;Overall, 110 COVID-19 patients were admitted. Thirty-two patients (29%) received CP; among them, 62.5% received at least one CP with high neutralizing antibody titers (≥1:160). Clinical improvement occurred within 28 days in 14 patients (43.7%) of the CP group vs. 48 patients (61.5%) in the non-CP group (hazard ratio (HR): 0.75 (95% CI: 0.41-1.37), p = 0.35). After adjusting for potential confounding factors, CP was not independently associated with time to clinical improvement (HR: 0.53 (95% CI: 0.23-1.22), p = 0.14). Additionally, the average treatment effects of CP, calculated using the inverse probability weights (IPW), was not associated with the primary outcome (-0.14 days (95% CI: -3.19-2.91 days), p = 0.93). Hospital mortality did not differ between CP and non-CP groups (31.2% vs. 19.2%, p = 0.17, respectively). Comparing CP with high neutralizing antibody titers to the other group yielded the same findings. (4) Conclusions: In this study of life-threatening COVID-19 patients, CP was not associated with time to clinical improvement within 28 days, or hospital mortality.

Background: Severe asthma is a major burden on health-economic resources; hence, knowing the epidemiology of these patients is important in planning and provision of asthma care. In addition, identifying and managing the comorbidities helps improve symptoms and reduce associated morbidity and mortality., Objectives: Epidemiology of difficult asthma has not been well studied in the Middle East, so in this study, we present the demographic and clinical characteristics of severe asthma in the United Arab Emirates (UAE)., Methods: We retrospectively reviewed the notes of severe asthma patients attending three tertiary care hospitals between May 2015 and December 2019. Data on baseline demographics, asthma characteristics, treatment, and comorbidities were collected., Results: We reviewed the notes of 458 patients (271 females and 187 males) that fulfilled the 2019 Global Initiative for Asthma guidelines for the diagnosis of severe asthma. The mean age was 47.7 (standard deviation 17.2) years. Males had significantly higher asthma control test scores (17.9 vs. 16, P = 0.01) and mean blood eosinophils (0.401 vs. 0.294, P <0.01) than females. The most common comorbidity observed was allergic rhinitis (52.2%) followed by gastroesophageal reflux disease (27.1%). In total, 109 (23.8%) patients were on biological therapies with most patients being on omalizumab and dupilumab (29 and 18 patients, respectively). Most patients were nonsmokers (97.2%), and majority were of TH2-high phenotype (75.7%)., Conclusions: In this first report of severe asthma characteristics in the UAE, we found a pattern of female preponderance and most patients having a Th2-high phenotype. The findings are likely to help optimize asthma care in the region in the era of biologic therapies., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Thoracic Medicine.)

Introduction: The COVID-19 pandemic dramatically disrupts health care around the globe. The impact of the pandemic on chronic urticaria (CU) and its management are largely unknown., Aim: To understand how CU patients are affected by the COVID-19 pandemic; how specialists alter CU patient management; and the course of CU in patients with COVID-19., Materials and Methods: Our cross-sectional, international, questionnaire-based, multicenter UCARE COVID-CU study assessed the impact of the pandemic on patient consultations, remote treatment, changes in medications, and clinical consequences., Results: The COVID-19 pandemic severely impairs CU patient care, with less than 50% of the weekly numbers of patients treated as compared to before the pandemic. Reduced patient referrals and clinic hours were the major reasons. Almost half of responding UCARE physicians were involved in COVID-19 patient care, which negatively impacted on the care of urticaria patients. The rate of face-to-face consultations decreased by 62%, from 90% to less than half, whereas the rate of remote consultations increased by more than 600%, from one in 10 to more than two thirds. Cyclosporine and systemic corticosteroids, but not antihistamines or omalizumab, are used less during the pandemic. CU does not affect the course of COVID-19, but COVID-19 results in CU exacerbation in one of three patients, with higher rates in patients with severe COVID-19., Conclusions: The COVID-19 pandemic brings major changes and challenges for CU patients and their physicians. The long-term consequences of these changes, especially the increased use of remote consultations, require careful evaluation., (© 2020 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Background: Pulmonary radiological findings of the novel coronavirus disease 2019 (COVID-19) have been well documented and range from scattered ground-glass infiltrates in milder cases to confluent ground-glass change, dense consolidation, and crazy paving in the critically ill. However, lung cavitation has not been commonly described in these patients. The objective of this study was to assess the incidence of pulmonary cavitation in patients with COVID-19 and describe its characteristics and evolution., Methods: We conducted a retrospective review of all patients admitted to our institution with COVID-19 and reviewed electronic medical records and imaging to identify patients who developed pulmonary cavitation., Results: Twelve out of 689 (1.7%) patients admitted to our institution with COVID-19 developed pulmonary cavitation, comprising 3.3% (n = 12/359) of patients who developed COVID-19 pneumonia, and 11% (n = 12/110) of those admitted to the intensive care unit. We describe the imaging characteristics of the cavitation and present the clinical, pharmacological, laboratory, and microbiological parameters for these patients. In this cohort six patients have died, and six discharged home., Conclusion: Cavitary lung disease in patients with severe COVID-19 disease is not uncommon, and is associated with a high level of morbidity and mortality.