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This article explores the relationship between basophil and eosinophil recruitment to skin lesions and autoimmune chronic spontaneous urticaria (CSU). The study reveals that CSU patients have increased levels of basophils and eosinophils in both lesional and non-lesional skin compared to healthy individuals. The numbers of these cells in the lesional skin are linked to disease activity and quality of life impairment. Furthermore, patients with low total IgE levels have higher levels of basophils and eosinophils in their lesional skin. Another study examines the connection between basophil and eosinophil counts in the blood and skin of CSU patients. It finds that patients with serum autoreactivity, indicated by a positive basophil activation test (BAT+), have higher levels of eosinophils in their skin lesions compared to BAT- patients. Additionally, BAT+ patients have lower blood counts of basophils and eosinophils, suggesting that their infiltration into the skin reduces their levels in the blood. The study emphasizes the need for further research on the role of these cells in CSU and the development of therapies targeting their recruitment and effects. [Extracted from the article]

Urticarial vasculitis (UV) is a rare and difficult-to-treat, small-vessel leukocytoclastic vasculitis presenting with recurrent long-lasting wheals. So far, no guidelines and treatment algorithms exist that could help clinicians with the management of UV. In this review, we describe evidence on systemic treatments used for UV and propose a clinical decision-making algorithm for UV management based on the Urticarial Vasculitis Activity Score assessed for 7 days (UVAS7). Patients with occasional UV-like urticarial lesions and patients with UV with skin-limited manifestations and/or mild arthralgia/malaise (total UVAS7 ≤7 of 70) can be initially treated using the step-wise algorithm for chronic urticaria including second-generation H1-antihistamines, omalizumab, and cyclosporine A. Patients with UV with more severe symptoms (UVAS7 >7), especially those with hypocomplementemic UV, may require a multidisciplinary approach, particularly if underlying diseases, for example, systemic lupus erythematosus, cancer, or infection, are present. Immunomodulatory therapy is based on clinical signs and symptoms, and the drug availability and safety profile, and includes systemic corticosteroids, dapsone, hydroxychloroquine, anti-interleukin-1 agents, and other therapies. The level of evidence for all UV treatments is low. Prospective studies with current and novel drugs are needed and could provide further insights into UV pathogenesis and treatment. [ABSTRACT FROM AUTHOR]

Introduction: There is a significant prevalence of chronic spontaneous urticaria (CSU) in children across the globe. Some children with CSU do not achieve disease control with first-line antihistamine treatment and may need anti-IgE therapy with omalizumab. Recently, several novel treatment options, including dupilumab and BTK inhibitors, showed promising results in the treatment of antihistamine-refractory CSU in adults. However, information regarding their use in pediatric CSU is scarce, and most data is extrapolated from adult studies., Areas Covered: The review highlights the evidence on the use of mAbs and small-molecule inhibitors in pediatric CSU and aims to bridge the knowledge gaps and highlight unmet needs., Expert Opinion: Omalizumab is approved for allergic asthma patients aged ≥6 years, and some experience with omalizumab in children with CSU at this age has been published. However, approximately 5-10% of pediatric CSU patients may show insufficient response to omalizumab, necessitating other therapies. The available information on the off-label use of biologics other than omalizumab in children is limited to case reports. No data is available for other new therapies.

Background: CRUSE® is an app that allows patients with chronic spontaneous urticaria (CSU) to monitor their daily disease activity through the use of visual analogue scales (VASs). We aimed to determine the concurrent validity, reliability, responsiveness and minimal important difference (MID) of CRUSE® VASs., Methods: We evaluated the properties of three daily VASs: VAS for how much patients were affected by their CSU ('VAS urticaria'), VAS for the impact of urticaria on work/school productivity ('VAS productivity') and the VAS of EQ-5D. Concurrent validity was assessed by measuring the association between each VAS and the Urticaria Activity Score (UAS). Intra-rater reliability was determined based on the data of users providing multiple daily questionnaires within the same day. Test-retest reliability and responsiveness (ability to change), respectively, were tested in clinically stable and clinically unstable users. MIDs were determined using distribution-based methods., Results: We included 5938 patients (67,380 days). Concurrent validity was high, with VAS urticaria being more strongly associated with the UAS score than the remaining VASs. Intra-rater reliability was also high, with intraclass correlation coefficients (ICC) being above 0.950 for all VASs. Moderate-high test-retest reliability and responsiveness were observed, with reliability ICC being highest for VAS EQ-5D and responsiveness being highest for VAS urticaria. The MID for VAS urticaria was 17 (out of 100) units, compared to 15 units for VAS productivity and 11 units for VAS EQ-5D., Conclusion: Daily VASs for CSU available in the CRUSE® app display high concurrent validity and intra-rater reliability and moderate-high test-retest reliability and responsiveness., (© 2024 European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Background: Initiatives aiming to assess the impact of rare diseases on population health might be hampered due to the complexity of disability-adjusted life years (DALYs) estimation. This study aimed to give insight into the epidemiological data sources and methodological approaches used in studies that estimated DALYs for chronic non-communicable rare diseases (CNCRD), and compare its results., Methods: A literature strategy was developed for peer-review search in Embase and Medline, and also performed on grey literature databases and population health and/or rare disease-focused websites. We included studies that determined the burden of CNCRD listed on the Orphanet's and/or the Genetic and Rare Diseases information center (GARD) websites. We excluded communicable and occupational diseases, rare cancers, and cost-effectiveness/benefit studies. Two researchers independently screened the identified records and extracted data from the final included studies. We used the Guidelines for Accurate and Transparent Health Estimates Reporting (GATHER) statement to assess the quality of reporting of the included studies. The data synthesis depicted the studies' characteristics, their distribution by geographic coverage and the group of disease(s) they focused on, the methods and data input sources used and estimated DALY per case., Results: In total, 533 titles were screened, and 18 studies were included. These studies covered 19 different CNCRDs, of which most fell in the disease category "Diseases of the nervous system". Diverse methodological approaches and data input sources were observed among burden of CNCRD studies. A wide range of DALY per case was observed across the different studies and diseases included., Conclusions: A low number of burden of CNCRD studies was observed and most estimates resulted from multi-country studies, underlining the importance of international cooperation to further CNCRD research. This study revealed a lack of epidemiological data and harmonization of methods which hampers comparisons across burden of CNCRD studies., (© 2024. The Author(s).)