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.
Oral Abstract Session 1: RhinitisO01 Long-term follow-up of local allergic rhinitis patientsIbon Eguiluz Gracia, Carmen Rondón, Paloma Campo, Ana Prieto, Lina Mayorga, Luisa Galindo, Ana Molina, Miguel Blanca, Maria Jose TorresHospital Regional Universitario de Malaga and IBIMA, Málaga, Spain Correspondence: Ibon Eguiluz Gracia - iboneguiluz@gmail.com Clinical and Translational Allergy 2017, 7(Suppl 3):O01 Introduction: There are few data available regarding natural history of local allergic rhinitis (LAR). We previously reported the results of the first 5-years of follow-up observing a similar rate of development of systemic allergic rhinitis (AR) in both LAR patients and healthy controls. Objective: To explore the natural history of a population with LAR and the development of AR and comorbidities over a 10 year period. Methods: A cohort of 194 patients with LAR of recent onset (<18 months) and 130 age- and sex-matched healthy controls were prospectively evaluated in a 10-year follow-up study (2005–2016). All participants provided informed consent and ethic committee of the hospital approved the study. Clinical-demographic questionnaire, spirometry, SPT and specific IgE (sIgE) to aeroallergens were evaluated yearly. Nasal allergen provocation tests (NAPT) with D. pteronyssinus (DP), Alternaria, Olea europea, and grass pollen were performed at baseline, and after 5 and 10 years. Results: A total of 151 patients (78%) and 90 controls (69%) completed the study. At baseline, most patients had moderate-to-severe persistent-perennial rhinitis. Conjunctivitis (52%) and asthma (19%) were the main comorbidities, and DP the most frequent sensitizing aeroallergen (51.1%). During the 10 years of evaluation 21 new cases of asthma (12.5%, P = 0.007) and 17 new cases of conjunctivitis (10%, P = 0.067) were diagnosed. After 10 years of evolution a similar rate of development of AR was detected in patients and healthy controls (11.3 vs 10%, P = 0.761). In 5 patients, conversion to systemic atopy occurred in the last year of evaluation (3%). Conclusions: LAR is a well-differentiated clinical entity with a low rate of development of systemic atopy. This study was funded by the Institute of Health “Carlos III” of the Spanish Ministry of Economy and Competitiveness through the RETICS ARADyAL (RD16/0006/0001) and the FIS PI14/00864. Keywords: Local allergic rhinitis, Allergic rhinitis, SensitizationO02 ILC2-activation aggravates Th2-dependent nasal inflammation in miceTaiyo Morikawa1, Ayumi Fukuoka1, Kazufumi Matsushita1, Shigeharu Fujieda2, Tomohiro Yoshimoto1 1Hyogo College of Medicine, Nishinomiya, Hyogo, Japan; 2University of Fukui, Fukui, Japan Correspondence: Taiyo Morikawa - taiyo1125@hotmail.co.jp Clinical and Translational Allergy 2017, 7(Suppl 3):O02 Introduction: It is well known that T helper (Th) 2 cells and group 2 innate lymphoid cells (ILC2s) contribute to allergic diseases. However, their exact role and relationship in nasal allergic disorders is unclear. We sought to investigate the cooperation of Th2 cells and ILC2s in a mouse model of nasal allergic disorder. Methods: To differentially activate Th2 cells and/or ILC2s in nasal mucosa, mice were intranasally administered ovalbumin (OVA) antigen, papain, an ILC2-activatior, or both for 2 weeks. Epithelial thickness and number of eosinophils in the nasal mucosa were evaluated at 24 h after the final challenge. Results: Intranasal administration of OVA and papain preferentially activated Th2 cells and ILC2s, respectively, in the nose. Both OVA and papain increased the nasal epithelial thickness and number of eosinophils, and their coadministration significantly enhanced the symptoms. ILC2- and Rag2-deficient mice showed a partial decrease in OVA-plus-papain-induced nasal epithelial thickening and eosinophilia. Interleukin (IL)-33- and ST2-deficient mice showed decreased OVA-plus-papain-induced, but not OVA-alone-induced nasal epithelial thickening and eosinophilia. IL-5 induced eosinophilia only, but IL-13 contributed to both nasal epithelial thickening and eosinophilia. Conclusions: IL-33/ST2-pathway-mediated ILC2 activation exacerbated additively Th2-cell-induced nasal type 2 inflammation. Furthermore, IL-13, but not IL-5, contributes to exacerbation of nasal type 2 inflammation. Keywords: Nasal allergy, Th2 cells, Group 2 innate lymphoid cells, IL-33, IL-13O03 Allergen endotoxins induce non-IgE-mediated nasal hypersensitivity in mice via monocyte/macrophage-dependent pathwayTomohiro Yoshimoto, Naruhito Iwasaki, Kazufumi MatsushitaHyogo College of Medicine, Nishinomiya, Japan Correspondence: Tomohiro Yoshimoto - tomo@hyo-med.ac.jp Clinical and Translational Allergy 2017, 7(Suppl 3):O03 Introduction: Allergen-mediated cross-linking of IgE on mast cells/basophils is a well-recognized trigger for type-1 allergic diseases such as allergic rhinitis (AR). However, allergens may not be the only trigger for AR, and several allergic-like reactions are induced by non-IgE-mediated mechanisms. Here, we describe a novel non-IgE-mediated, endotoxin-triggered nasal type-1-hypersensitivity reaction in mice. Methods: To investigate whether endotoxin affects sneezing responses, mice were intraperitoneally immunized with ovalbumin (OVA), and then nasally challenged with endotoxin-free or endotoxin-containing OVA. To investigate the role of T cells and mechanisms of the endotoxin-induced response, mice were adoptively transferred with in vitro differentiated OVA-specific Th2 cells, and then nasally challenged with endotoxin-free or endotoxin-containing OVA. Immediately after each nasal challenge, the frequency of sneezing was counted for 10 min. The mice were sacrificed 24 h after the final nasal challenge, and noses were dissected for analyzing infiltrating inflammatory cells. Results: Endotoxin-containing, but not endotoxin-free, OVA elicited sneezing responses in mice independent from IgE-mediated signaling. OVA-specific Th2 cell adoptive transfer to mice demonstrated that local activation of antigen-specific Th2 cells was required for the response. The Toll-like receptor 4-MyD 88-signaling pathway was indispensable for endotoxin-containing OVA-elicited rhinitis. In addition, lipopolysaccharide directly triggered sneezing responses in OVA-specific Th2-transferred and nasally endotoxin-free OVA-primed mice. Although an antihistamine, diphenhydramine, suppressed sneezing responses, mast-cell/basophil-depleted mice had normal sneezing responses to endotoxin-containing OVA. Clodronate treatment abrogated endotoxin-containing OVA-elicited rhinitis, suggesting the involvement of monocytes/macrophages in this response. Conclusions: Antigen-specific nasal activation of CD4+ T cells followed by endotoxin exposure induces mast cell/basophil-independent histamine release in the nose that elicits sneezing responses. Thus, environmental or nasal residential bacteria may exacerbate AR symptoms. In addition, this novel phenomenon might explain currently unknown mechanisms in non–IgE-mediated allergic disorders, such as non-IgE-mediated gastrointestinal food allergy in infants. Keywords: Non-allergic rhinitis, Endotoxin, Histamine, T cells, Monocytes/macrophages
Immunoglobulin E (IgE) and its receptor, FcɛRI, importantly contribute to the pathophysiology of chronic spontaneous urticaria (CSU). Recent findings point to a possible role of total IgE as a marker of CSU disease activity, endotypes, and responses to treatment. The evidence in support of total IgE included in the diagnostic workup of patients with CSU has not yet been reviewed.Publications were searched via PubMed. The search terms used were "chronic urticaria" and "total IgE." Studies were screened by titles and abstracts, and 141 were used in the review.CSU patients frequently had elevated total IgE serum levels (up to 50%), but normal or very low total IgE levels also occurred. High total IgE may represent high disease activity, longer disease duration, high chance of responding to omalizumab treatment, quick relapse after stopping omalizumab, and lower chance of responding to cyclosporine. Low IgE, in contrast, may suggest Type IIb autoimmune CSU, poor response to treatment with omalizumab and a better chance to benefits from cyclosporine treatment. Furthermore, IgE in different CSU cohorts may have different physicochemical properties that could explain differences in treatment responses to IgE-directed therapies.The results of our review suggest that total IgE is a valuable marker for CSU, and we recommend its assessment in the routine diagnostic workup of CSU patients.
Here we present a case of a patient with breathlessness and cough admitted to the COVID ward. Chest radiography demonstrates findings consistent with lobar collapse, giving rise to the 'Luftsichel sign'. This sign has been described in the literature and highlights the importance of recognition and prompt further investigation.
The correct classification of an adverse drug reaction (ADR) as allergy (immunological) or intolerance (non-immunological) has important clinical implications. The aim of this study was to examine the ability of health professionals to discriminate between allergy and intolerance, classify the severity of the ADR and degree of contraindication.Health professionals were presented ten 'real-life' ADR scenarios using an online questionnaire and asked to: categorise the reaction as allergy or intolerance, rate the severity of the reaction and judge the level of contraindication of the causative drug. The number and proportion of responses were calculated for each of the cases presented and associations between classification of reaction type, severity and level of contraindication were examined.A total of 394 responses were received. Overall 59.0% (SD 28.9) correctly categorised the cases, 60.8% (SD 16.8) classified the severity correct, and less than half (44.7%, SD 28.6) correctly identified the level of contraindication. The proportion of health professionals correctly answering the type, severity and level of contraindication for the allergy case was significantly higher (p < 0.0001) by comparison to the intolerance cases (type: 56.6% ± 33.1; severity: 57.3 ± 11.9; level of contraindication: 38.5 ± 19.9).Health professionals have suboptimal understanding of classification of ADRs. Strategies are required to strictly avoid re-exposure of patients to drugs which carry an increased risk of inducing a dangerous reaction, whilst minimising the avoidance of drugs which are of minimal risk or allowing the use of low-risk drugs where the benefits may be significant.
Urticaria is a common skin condition encountered across various specialties in medicine, especially in dermatology and allergy/immunology practice. It has a heterogeneous presentation hence it is unsurprising that many skin conditions may be confused with urticaria. Urticaria may present as acute or chronic urticaria, the latter can be further categorised into chronic spontaneous and chronic inducible. In this article, we explore, explain, and summarise various skin lesions that are considered mimickers of urticaria, to promote understanding of each of the conditions highlighted, improve recognition, and reduce misdiagnosis.
Marking nut Semecarpus anacardium, so-called because it contains a pigment that has been used in the past to mark fabrics, is a known cause of contact hypersensitivity. It may be ingested as an ingredient of some traditional Hindi foods. We describe the first reported case of anaphylaxis to marking nut.
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