RESUMEN
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.
Asunto(s)
Angioedema , Consenso , Terminología como Asunto , Humanos , Angioedema/clasificación , Angioedema/diagnóstico , Abreviaturas como Asunto , Técnica DelphiRESUMEN
PURPOSE: The importance of carbohydrates in anaphylaxis has been described with some foods. The current work intends to obtain clinical and immunological evidence of the importance of the O-glycans for IgE binding activity in anaphylactic reactions due to Helix aspersa (HA) ingestión and Artemisia vulgaris (AV) exposition. METHODS: The studio focused on two cases of IgE-mediated anaphylaxis induced by snail ingestion in patients with underlying rhino-conjunctivitis and asthma due to AV. We performed on both patients: skin prick tests ( SPTs) with HA and AV and with a battery of aeroallergen, controlled nasal challenge and specific IgE to HA and AV, ImmunoCAP ISAC®, and a differential pattern of IgE recognition with SDS-PAGE Immunoblotting (SDSI) when these allergens have suffered an O-deglycosylation procedure. RESULTS: The patients showed positive results in SPTs, nasal challenges, and serum-specific IgE against HA and AV. In patient 1, the SDSI detected several IgE-binding proteins in AV with a molecular mass of 22, 24, and 44 kDa, whereas a band of 12 kDa was detected in HA. On the other hand, patient 2's serum revealed an IgE-binding zone between 75 and 20 kDa in the AV and a band of 24 kDa in the HA. When glycans were removed, patient 1's serum only revealed the AV's 22 and 24 kDa bands, whereas patient 2's serum did not detect any IgE-reactive protein in the HA. CONCLUSIONS: Our data suggest that O-glycosylation can be relevant in patients with anaphylaxis due to snails and allergy to Artemisia vulgaris. This new entity representing cross-reactivity between AV and HA could be named Snail-Artemisia Syndrome.
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Anafilaxia , Artemisia , Rinitis Alérgica Estacional , Humanos , Anafilaxia/diagnóstico , Anafilaxia/etiología , Carbohidratos , Polisacáridos , Inmunoglobulina ERESUMEN
BACKGROUND: There is a need for a disease-specific instrument for assessing health-related quality of life in adults with hereditary angioedema due to C1 inhibitor deficiency, a rare, disabling and life-threatening disease. In this paper we report the protocol for the development and validation of a specific questionnaire, with details on the results of the process of item generation, domain selection, and the expert and patient rating phase. METHODS/DESIGN: Semi-structured interviews were completed by 45 patients with hereditary angioedema and 8 experts from 8 regions in Spain. A qualitative content analysis of the responses was carried out. Issues raised by respondents were grouped into categories. Content analysis identified 240 different responses, which were grouped into 10 conceptual domains. Sixty- four items were generated. A total of 8 experts and 16 patients assessed the items for clarity, relevance to the disease, and correct dimension assignment. The preliminary version of the specific health-related quality of life questionnaire for hereditary angioedema (HAE-QoL v 1.1) contained 44 items grouped into 9 domains. DISCUSSION: To the best of our knowledge, this is the first multi-centre research project that aims to develop a specific health-related quality of life questionnaire for adult patients with hereditary angioedema due to C1 inhibitor deficiency. A preliminary version of the specific HAE-QoL questionnaire was obtained. The qualitative analysis of interviews together with the expert and patient rating phase helped to ensure content validity. A pilot study will be performed to assess the psychometric properties of the questionnaire and to decide on the final version.
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Angioedemas Hereditarios/psicología , Proteínas Inactivadoras del Complemento 1/deficiencia , Psicometría/métodos , Calidad de Vida , Encuestas y Cuestionarios , Adolescente , Adulto , Anciano , Medicina Basada en la Evidencia , Femenino , Humanos , Entrevistas como Asunto , Masculino , Persona de Mediana Edad , Proyectos Piloto , Investigación Cualitativa , Reproducibilidad de los Resultados , Índice de Severidad de la Enfermedad , EspañaRESUMEN
BACKGROUND: Eosinophilic esophagitis (EoE) is an antigen-driven disease mediated by an abnormal immune Th2 response. OBJECTIVE: The objective of this article is to investigate genes associated with regulating immune responses leading to disease susceptibility. METHODS: Twenty-seven tag single nucleotide polymorphisms (tSNPs) selected in five candidate genes (TLR3, TLR4, FOXP3, FLG and TSLP) were genotyped in 218 EoE patients and 376 controls. Skin prick tests were carried out in EoE patients with a panel of 17 aeroallergens and 22 plant- and animal-derived foods. RESULTS: Five tSNPs located in the TSLP locus and one tSNP located in the TLR3 locus were significantly associated with EoE. The interactions between TLR3 and TSLP loci were analyzed. TLR3+/TSLP- and TLR3-/TSLP+ individuals showed a significantly reduced susceptibility to EoE compared to TLR3-/TSLP- individuals (OR = 0.66, p = 0.036 and OR = 0.23, p = 0.00014, respectively). Likewise, TLR3+/TSLP+ individuals showed the most decreased susceptibility of developing EoE (OR = 0.16, p = 0.0001). However, the interaction gain attributed to the combination of both genes was negative (IG = -4.52%), which indicated redundancy or independent effect. Additionally, TLR3 locus was found to be associated with aeroallergen and food sensitization in EoE patients (OR = 9.67, pc = 0.025 and OR = 0.53, pc = 0.048, respectively). CONCLUSION: TLR3 constitutes a novel genetic susceptibility locus for developing EoE, and the effects would be independent of TSLP.
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Antiinflamatorios no Esteroideos/efectos adversos , Inhibidores de la Ciclooxigenasa 2/efectos adversos , Erupciones por Medicamentos/etiología , Pirazoles/efectos adversos , Piridinas/efectos adversos , Sulfonamidas/efectos adversos , Sulfonas/efectos adversos , Adulto , Anciano , Celecoxib , Hipersensibilidad a las Drogas , Etoricoxib , Femenino , Humanos , MasculinoRESUMEN
The aim of this study was to establish the clinical types of food reactions and the food sensitization profile in an adult cohort of patients with (EoE). We have prospectively analyzed the food allergen sensitization profile using skin prick test (SPT) and atopy patch test (APT) to 22 plant and animal-derived foods in 19 adult patients with biopsy-proven EoE. For each patient the following data was collected: age, sex, atopic disease status, and clinical characteristics of the EoE. A total of 123 food sensitizations were detected by SPT and 45 food sensitizations by patch testing in 18 patients (94.8%). Thirty-five double- positive (SPT+, APT+) responses against the same food were detected in 15 patients (78.9%). We have also identified 11 clinical episodes clearly suggestive of oral allergy syndrome, urticaria and/or anaphylaxis after intake of some specific foods. All cases showed positive SPT to the offending food (Type 1 food reactors). 68 clinically apparent episodes of EoE related with specific foods were detected in our patients. In 40 instances (58.8%) the foods involved in developing EoE symptoms showed a positive SPT and/or APT results (Type 2). 155 food sensitizations were identified in 18 patients (94.7%) using SPT and/or APT neither related with clinically apparent episodes of EoE nor with clinical episodes suggestive of oral allergy syndrome, urticaria and/or anaphylaxis after food intake (Type 3). In conclusion, the patients with EoE can be allocated into 3 different groups of food reactors, with well-defined clinical and biological characteristics.
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Anafilaxia/etiología , Esofagitis Eosinofílica/etiología , Hipersensibilidad a los Alimentos/complicaciones , Adolescente , Adulto , Anciano , Esofagitis Eosinofílica/inmunología , Femenino , Hipersensibilidad a los Alimentos/inmunología , Humanos , Masculino , Persona de Mediana Edad , Pruebas del Parche/métodos , Estudios Prospectivos , Pruebas Cutáneas/métodos , Urticaria/etiología , Adulto JovenRESUMEN
BACKGROUND: Endogenous airway acidification evaluated as pH in exhaled breath condensate (EBC) has been described in patients with chronic cough. Proton pump inhibitors improve gastro-oesophageal reflux (GOR)-associated cough. METHODS: We examined pH levels in EBC and capsaicin cough response in 13 patients with chronic cough (mean age 41 years, SD 9) associated with GOR before and after omeprazole treatment (40 mg/day for 14 days) and its relationship with clinical response. RESULTS: Omeprazole abolished symptoms associated with GOR. Patients with chronic cough had an EBC pH of 8.28 (SD 0.13) prior to treatment but this did not change with omeprazole treatment. There was a significant improvement in the Leicester Cough Questionnaire symptom scores from 80.8 points (SD 13.2) to 95.1 (SD 17) (p = 0.02) and in a 6-point scale of cough scores, but there was no change in capsaicin cough response. CONCLUSION: An improvement in GOR-associated cough was not associated with changes in EBC pH or capsaicin cough response. These parameters are not useful markers of therapeutic response.