Clinical Reviews in Allergy & Immunology最新文献

Chronic spontaneous urticaria (CSU) is characterized by recurrent wheals and/or angioedema without an identifiable trigger. Despite advances in therapy-including biologics such as omalizumab-suboptimal treatment adherence remains a major challenge, often resulting in poor symptom control and diminished quality of life. The objective of this study is to evaluate the existing literature on adherence in CSU and identify evidence-based strategies for improving long-term treatment engagement. A systematic literature search of PubMed and EMBASE was performed for articles published between 2000 and 2024 using the terms "chronic spontaneous urticaria," "chronic urticaria," "chronic idiopathic urticaria," "compliance," and "adherence." Eligible studies reported original data on adherence-related factors in CSU. Each study was categorized to World Health Organization (WHO) adherence dimensions: social/economic, healthcare system, condition-related, therapy-related, and patient-related. Risk of bias assessment was conducted for each study included in the final selection. The search followed PRISMA guidelines, and the protocol was registered with PROSPERO (ID: CRD42024627967). Twenty-one studies (totaling 18,500 patients) met inclusion criteria. Common barriers included lack of preventative medication use, inconvenience, forgetfulness, dissatisfaction with healthcare providers, and logistical challenges in accessing in-office biologic administration. Patients previously treated with immunosuppressants had a poorer response to omalizumab, which may contribute to nonadherence due to perceived lack of efficacy. External factors such as the COVID-19 pandemic also contributed to nonadherence by reducing clinic visits and access to specialist referrals. Higher education level and employment were significantly associated with improved adherence. Proposed strategies include simplifying treatment regimens, enhancing patient education about CSU chronicity, providing telehealth or home medication administration options, and offering financial or social support programs. Multiple interrelated barriers contribute to treatment nonadherence in patients with CSU, underscoring the need for multifaceted, patient-centered interventions. Clear communication, simplified regimens, and supportive resources may enhance adherence and help achieve improved long-term clinical outcomes.

Furry animals are familiar companions in modern society. Despite multiple beneficial roles in economic and social contexts, they can be the source of allergenic compounds. Moreover, research indicates that these allergens could be detected even in households where animals are not present. Consequently, the risk of asthma exacerbation is increased. Furthermore, accurately diagnosing a genuine allergy to furry animals remains a significant challenge for medical practitioners. Therefore, this review aims to gather and summarize valid information regarding three main groups of allergens associated with furry animals, including lipocalins, serum albumins, and secretoglobins. In this manuscript, we clarify the molecular structure of allergens, discuss cross-reactions between them, and highlight their clinical importance. We also outline the diagnostic techniques for furry animal allergy, as well as novel, emerging therapies. Additionally, we discuss the occupational risks of allergies for both laboratory workers and cattle farmers.

The aim of this study is to analyze data from all cases of Susac syndrome that have been published in the literature. This study was conducted in conformity with the PRISMA statement. A systematic review and survival analysis was performed for overall survival (OS) and progression-free survival (PFS). A total of 435 patients (males: 32.6%, n = 142), derived from 176 studies, were included in the analysis. The median age at diagnosis was 35 years (n = 433/435; range 7-69). Neurological symptoms were the most frequent (87.4%, n = 380/435). Among audio-vestibular symptoms (84.3%, n = 365/433), hearing loss was the most common manifestation (87.9%, n = 327/372). Branch retinal artery occlusions (BRAOs) were observed in 91.6% of patients (n = 261/285), while ophthalmological symptoms were reported in 78.9% (n = 288/365). The complete triad at onset was present in only 20.1% (n = 61/304) of cases. Complete recovery without sequelae was achieved in 59.5% (n = 44/74) of patients which reported neurological involvement, 39.7% (n = 27/68) of those with ophthalmological symptoms, and 17.7% (n = 11/62) of those with audio-vestibular symptoms. Partial recovery was observed in 36.5% (n = 27/74), 50.0% (n = 34/68), and 38.7% (n = 24/62) of patients, respectively. In a subgroup of 123 patients with available follow-up data (median duration: 12 months), 82.1% (n = 101) remained stable with controlled disease, 12.2% (n = 15) experienced a relapse, and 5.7% (n = 7) died. OS at 1 and 3 years was 94% (95% CI 88-97%). PFS was 89% at 1 year (95% CI: 80-94%) and 83% at 3 years (95% CI: 69-91%). Susac syndrome presents a significant clinical challenge. While presentations and outcomes are very heterogeneous, mortality is uncommon. The potential for relapse and long-term sequelae highlights the need for greater awareness and deeper understanding of the disease.

Asthma is a major chronic non-communicable respiratory disease, affecting over 300 million individuals globally, with an adult prevalence of 4.3%. Despite advances in individualized treatment, a subset of patients, particularly those with non-type 2 (non-T2) asthma, fails to achieve optimal disease control. Non-T2 asthma, characterized by steroid resistance and heterogeneous immune responses, remains a therapeutic challenge. Emerging evidence suggests that circadian rhythm disruption may modulate key pathological processes relevant to non-T2 asthma, including immune imbalance, epithelial barrier dysfunction, and impaired glucocorticoid sensitivity. This review investigates evidence of an association between circadian clock dysfunction and non-type 2 asthma pathogenesis, and proposes a hypothesis-driven framework to guide future studies. Chronotherapeutic strategies and clock-targeted interventions may offer promising avenues for future research and individualized treatment.

Objectives: This study aimed to use Bayesian network meta-analysis to compare the efficacy and safety of biologics for systemic lupus erythematosus (SLE). A comprehensive and systematic search of electronic databases (PubMed, Medline, Cochrane Library, EMBASE, Web of Science, CNKI, and WanFang Data) was conducted from 2014 to September 2024. Our study only included randomized controlled trials with full articles that enrolled adult SLE patients treated with biologics, in comparison with standard therapy. The primary efficacy endpoints were SLE Responder Index 4 (SRI4) and BICLA (BILAG-Based Composite Lupus Assessment). The safety endpoints were adverse events (AEs) and serious adverse events (SAEs). R 4.4.3 and RStudio were used to conduct the network meta-analysis. RevMan 5.4 was used to assess the included literature. 29 randomized controlled trials with a total of 13,712 patients met the inclusion criteria. The network meta-analysis indicated that compared with standard therapy, telitacicept (OR 5.2, 95% CI 1.4-20.0) demonstrated superior efficacy in achieving SRI4 response, deucravacitinib (OR 1.6, 95% CI 1.0-2.5), and anifrolumab (OR 1.6, 95% CI 1.3-2.0) all exhibited significant BICLA response in moderate-to-severe SLE patients. Regarding safety, it was observed that there were no significant statistical differences among the various treatment options. Cluster analysis revealed that deucravacitinib exhibited the best efficacy-safety profile. Deucravacitinib suggested a favorable profile between efficacy and safety. Telitacicept showed the most pronounced improvement in SRI-4 response, but was associated with higher rates of AEs and SAEs, whereas anifrolumab and deucravacitinib displayed advantages in reducing SAEs. For patients with elevated baseline IFN signatures, anti-type I interferon biologics such as anifrolumab and sifalimumab are recommended to maximize clinical benefits. The reliance on indirect comparisons necessitates cautious interpretation of these findings, so further research should prioritize direct head-to-head trials to validate the efficacy and safety profiles of these biologics.

Aquaporins (AQPs) are transmembrane proteins that facilitate the transport of water and small solutes across cell membranes. Their expression in organs such as the skin, lungs, gastrointestinal tract, and immune system has been implicated in the pathophysiology of various allergic diseases. Emerging evidence suggests that AQPs, particularly AQP1, AQP3, AQP4, AQP5, AQP7, and AQP9, are differentially regulated during allergic responses, contributing to symptoms such as mucosal hypersecretion, edema, and skin dryness. In atopic dermatitis, AQP3 overexpression correlates with increased transepidermal water loss, while in food allergy models, downregulation of AQP4 and AQP8 is associated with allergic diarrhea. In asthma, altered expression of AQP1, AQP3, and AQP5 has been linked to airway inflammation, eosinophil migration, and mucus production. The cAMP/CREB and NFκB signaling pathways have been identified as key regulators of AQP5 expression, providing potential therapeutic targets. Several experimental studies using herbal extracts have demonstrated anti-inflammatory effects mediated through upregulation of AQP1 and AQP5 and suppression of proinflammatory cytokines. A proposed integrative model suggests that AQPs participate in both the sensitization and effector phases of the allergic response, influencing antigen presentation, immune cell migration, and mediator release. Despite promising preclinical findings, human studies remain limited. Further clinical research is warranted to validate AQPs as biomarkers and therapeutic targets in allergic diseases, especially considering the increasing global prevalence of these conditions.

As a key epigenetic regulator, E3 ubiquitin-ligase ubiquitin-like containing PHD and RING finger domains 1 (UHRF1) is essential for maintaining genomic stability. The aberrant activation of UHRF1 has been strongly implicated in cancer progression. Emerging evidence indicates that UHRF1 serves as a pivotal orchestrator of immune homeostasis. This review provides an overview of the impact of UHRF1 on both innate and adaptive immunity, highlighting its epigenetic and regulatory roles in the development and function of macrophages, T lymphocytes, and B lymphocytes. Crucially, the protective mechanisms of UHRF1 in autoimmune disorders while concurrently detailing its tumor-promoting functions are dissected. Finally, this review discusses the therapeutic challenges and future perspectives for targeting UHRF1 in autoimmune disorders and cancers.

With the acceleration of global urbanization, residential proximity to major roadways (RPMR) has been recognized as a significant threat to public health, while the association between road proximity and risks of allergic respiratory outcomes remains unclear. This study aims to evaluate the associations between RPMR and the risks of allergic respiratory outcomes. We conducted a systematic literature search for existing scientific literature from databases of PubMed, EMBASE, Web of Science, Cochrane, and Scopus. The study protocol was registered at PROSPERO (registration ID: CRD42024604182). Random effects models were applied to evaluate the associations between RPMR and the risks of allergic respiratory outcomes by calculating the pooled odds ratio (OR) and corresponding 95% confidence interval (CI). During the study periods, 55 eligible studies were included, comprising 373,320 participants. We found that a close RPMR (≤ 200 m) was associated with increased risks of asthma (OR = 1.23, 95% CI: 1.15, 1.31), wheezing (OR = 1.21, 95% CI: 1.12, 1.30), and rhinitis (OR = 1.22, 95% CI: 1.13, 1.32). In addition, we identified that the closer the RPMR, the higher the risks for allergic respiratory outcomes. The observed associations between RPMR and allergic respiratory outcomes were more pronounced among children and less urbanized areas than in adults and highly urbanized areas. Our study provides comprehensive evidence for the associations between RPMR and risks for allergic respiratory outcomes. The findings may contribute to the practical implications for urban planning and public health strategies to mitigate exposure to traffic-related pollution.

Inborn errors of immunity (IEIs) are traditionally viewed as monogenic disorders of the immune system, but mounting evidence indicates that they often have underappreciated impacts on the nervous system. We review the emerging intersection between IEIs and neurological diseases, spanning neurodevelopmental and neurodegenerative manifestations. We discuss how genetic overlaps between immunity and brain development-for example, defects in DNA repair, chromatin remodeling, or cytokine signaling-can lead to combined immunological and neurological phenotypes. Clinical data from patient cohorts highlight that a substantial subset of IEIs present with neurodevelopmental disorders (such as autism spectrum disorder, intellectual disability, or ADHD) and/or neurodegenerative diseases (such as progressive ataxia, motor regression, or cognitive decline). These comorbidities arise through diverse mechanisms, including direct roles of immune genes in neural development, the impact of chronic inflammation on the brain, and metabolic byproducts toxic to neural tissue. We illustrate these mechanisms with examples such as ataxia-telangiectasia (a DNA repair defect causing immunodeficiency and cerebellar degeneration) and DiGeorge syndrome (a developmental immunodeficiency often initially diagnosed as autism). The translational importance of these insights is profound-recognizing neurological involvement in IEIs can improve early diagnosis and multidisciplinary care, whereas a deeper understanding of immune-neural crosstalk opens avenues for novel therapies (such as targeted anti-inflammatory treatments or gene therapies that address both immune and neural dysfunction). By integrating immunology and neuroscience perspectives, this comprehensive review sheds light on the immune underpinnings of certain neurologic diseases and underscores the importance of collaborative management for patients at this complex interface.

Background: Inherited ARPC1B deficiency (ARPC1BD) is a rare autosomal recessive inborn error of immunity that manifests with allergic, infective, and autoimmune/inflammatory features. Only about three dozen patients with ARPC1BD have been reported in the literature thus far.

Methods: We describe 14 patients (ten families) with ARPC1BD from Nepal. Many of these patients have unique/rare clinical features that expand the phenotypic spectrum of ARPC1BD. The study included data on clinical-epidemiological features, immuno-hematological parameters, treatment, and outcome. Homozygosity mapping and haplotype analysis were used to evaluate the founder effect.

Results: We noted allergic/autoimmune and infective manifestations in all of our patients. Notable clinical features noted in our study include rheumatoid factor positive (RF+) chronic arthritis (mimicking RF+ juvenile idiopathic arthritis), significantly elevated anti-tissue transglutaminase antibody titers, generalized skin hyperpigmentation, distal phalangeal enlargement, frontal bone hypertrophy, hypertrophic skin scars, and hyperkeratosis. All cases harbored the founder splice-site variant c.64+2T>A in the ARPC1B gene. Long-term outcomes in our patients appeared worse than reported previously. Comparative phenotypic analysis showed significantly greater proportions of otitis, gastroenteritis, inflammatory bowel disease-like manifestations, and elevated IgA in patients with c.64+2T>A variant as compared to other variants. Homozygosity mapping (in eight probands) revealed that the gene/variant is contained within the only overlapping region of homozygosity (>1 Mb) across all autosomal chromosomes. Besides, the included probands share a similar haplotype around the said variant.

Conclusions: c.64+2T>A is a founder variant in Nepalese patients with ARPC1BD. This variant is associated with a severe clinical phenotype and diverse clinical complications.