The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update.

Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.

[Mexican consensus on the diagnosis and treatment of atopic dermatitis in adolescents and adults]. / Consenso mexicano para el diagnóstico y tratamiento de la dermatitis atópica en adolescentes y adultos.

BACKGROUND: The diagnostic approaches and therapeutic strategies of atopic dermatitis (AD) are generally inconsistent among physicians and health institutions. OBJECTIVE: To develop a consensus statement among experts to reduce the variations in practice regarding the diagnosis and treatment of patients ≥ 12 years with AD to improve their care. METHODS: Systematic literature search in PubMed and GREAT. With methodological support and using the Delphi method, a formal consensus was developed among 16 experts in Dermatology and Allergology, based on the current evidence and its applicability in the Mexican context. Apart from intense electronic communication, several issues of disagreement were discussed in two face-to-face meetings. RESULTS: The clinical experts reached consensus on 46 statements related to the definition, classification, diagnostic strategies and treatment of AD. For the diagnosis we suggest the Williams criteria and for severity scoring the SCORAD (by the doctor) and POEM (by the patient). In addition to general care and treatment education (workshops), we suggest four steps for treatment, depending on severity: 1. Topical treatment with anti-inflammatory agents (and systemic: antihistamines/antileukotrienes -low level evidence-) 2. Phototherapy, 3. Cyclosporin A and 4. Dupilumab, with the possibility of managing this biological earlier on if a fast effect is needed. In extrinsic AD we suggest evaluating the addition of allergen immunotherapy or an elimination diet, if there is an IgE-mediated respiratory or food allergy, respectively. CONCLUSION: The panel of experts reached consensus on relevant aspects of AD with a focus on the transcultural adaptation of recent evidence.

Antecedentes: Los abordajes diagnósticos y las estrategias terapéuticas de la dermatitis atópica generalmente son inconsistentes entre los médicos y entre las instituciones de salud. Objetivo: Consensar las opiniones de expertos para reducir las variaciones en la práctica respecto al diagnóstico y tratamiento de pacientes ≥ 12 años con dermatitis atópica para mejorar su cuidado. Métodos: Búsqueda sistemática de la literatura en PubMed y GREAT. Con apoyo metodológico y utilizando el método Delphi se desarrolló un consenso formal entre 16 expertos en dermatología y alergología, basándose en la evidencia actual y su aplicabilidad en el contexto mexicano. A parte de una comunicación electrónica intensa, se discutieron los puntos en desacuerdo en dos reuniones presenciales. Resultados: Los expertos clínicos alcanzaron consenso en 46 declaraciones relacionadas con la definición, clasificación, estrategias de diagnóstico y tratamiento de la dermatitis atópica. Para el diagnóstico sugerimos se usan los criterios de Williams y el SCORAD (por parte del médico) y POEM (por parte del paciente) para definir la gravedad. Aunado a cuidados generales y educación terapéutica, sugerimos cuatro pasos para tratamiento, según gravedad: 1. Manejo tópico con antiinflamatorio (y sistémico: antihistamínico/antileucotrieno ­evidencia reducida­) 2. Fototerapia, 3. Ciclosporina A y 4. Dupilumab, con la posibilidad de manejarlo antes si se necesita efecto rápido. En la dermatitis atópica extrínseca sugerimos agregar inmunoterapia con alérgenos o una dieta de eliminación si existe una alergia IgE-mediada, inhalatoria o alimentaria, respectivamente. Conclusión: El panel de expertos realizó consenso en aspectos relevantes de la dermatitis atópica con enfoque en la adaptación transcultural de evidencia reciente.

In the (sub)tropics allergic rhinitis and its impact on asthma classification of allergic rhinitis is more useful than perennial-seasonal classification.

BACKGROUND: Two different allergic rhinitis (AR) symptom phenotype classifications exist. Treatment recommendations are based on intermittent-persistent (INT-PER) cataloging, but clinical trials still use the former seasonal AR-perennial AR (SAR-PAR) classification. This study was designed to describe how INT-PER, mild-moderate/severe and SAR-PAR of patients seen by allergists are distributed over the different climate zones in a (sub)tropical country and how these phenotypes relate to allergen sensitization patterns. METHODS: Six climate zones throughout Mexico were determined, based on National Geographic Institute (Instituto Nacional de Estadística y Geografía) data. Subsequent AR patients (2-68 years old) underwent a blinded, standardized skin-prick test and filled out a validated questionnaire phenotyping AR. RESULTS: Five hundred twenty-nine subjects participated in this study. In the tropical zone with 87% house-dust mite sensitization, INT (80.9%; p < 0.001) and PAR (91%; p = 0.04) were more frequent than in the subtropics. In the central high-pollen areas, there was less moderate/severe AR (65.5%; p < 0.005). Frequency of comorbid asthma showed a clear north-south gradient, from 25% in the dry north to 59% in the tropics (p < 0.005). No differences exist in AR cataloging among patients with different sensitization patterns, with two minor exceptions (more PER in tree sensitized and more PAR in mold positives; p < 0.05). CONCLUSION: In a (sub)tropical country the SAR-PAR classification seems of limited value and bears poor relation with the INT-PER classification. INT is more frequent in the tropical zone. Because PER has been shown to relate to AR severity, clinical trials should select patients based on INT-PER combined with the severity cataloging because these make for a better treatment guide than SAR-PAR.

[ARIA Mexico 2014. Adaptation of the Clinical Practice Guide ARIA 2010 for Mexico. Methodology ADAPTE]. / ARIA México 2014. Adaptación de la Guía de Práctica Clínica ARIA 2010 para México. Metodología ADAPTE.

BACKGROUND: The global prevalence of allergic rhinitis is high. International Study of Asthma and Allergies in Childhood (ISAAC) Phase III reports a total estimated prevalence of 4.6% in Mexico. There is evidence based on allergic rhinitis Clinical Practice Guidelines (CPG), but its promotion, acceptance and application is not optimal or adequate in Mexico. OBJECTIVE: To generate a guideline for the treatment of allergic rhinitis and its impact on asthma by adaptating the 2010 ARIA Guideline to Mexican reality, through a transculturation process applying the ADAPTE methodology. PATIENTS AND METHOD: Using the ADAPTE Methodology, the original 2010 ARIA CPG recommendations were evaluated by the guideline development group (GDG) into which multiple medical specialities managing patients with allergic rhinitis were incoorporated. The GDG valorated the quality of 2010 ARIA, checked and translated key clinical questions. Moreover, the GDG adjusted recommendations, patient preferences and included comments in the context of the Mexican reality (safety, costs and cultural issues). To accomplish this, we ran Delphi panels with as many rounds as necessary to reach agreement. One extra question, not included in the original 2010 ARIA, on the use of Nasal Lavages for AR was created sustained by a systematic literature review. RESULTS: A total of 45 questions from the original 2010 ARIA were included and divided into six groups covering prevention, medical treatment, immunotherapy and alternative medicine to treat patients with allergic rhinitis with or without asthma. Most of the questions reached agreement in one or two rounds; one question required three rounds. CONCLUSIONS: An easy-to-use, adaptated, up-to-date and applicable allergic rhinitis guideline for Mexico is now available.

ANTECEDENTES: la prevalencia de rinitis alérgica en todo el mundo es alta. El Estudio Internacional de Asma y Alergias en la Niñez (ISAAC de International Study of Asthma and Allergies in Childhood) Fase III reporta una prevalencia estimada total en México de 4.6%. Existen guías de práctica clínica basadas en evidencia de rinitis alérgica, pero su promoción, aceptación y validez no son óptimas ni adecuadas para México. OBJETIVO: generar una guía de tratamiento de la rinitis alérgica y su repercusión en el asma adaptando la guía ARIA 2010 a la realidad mexicana mediante un proceso de transculturización, por medio de la metodología ADAPTE. MATERIAL Y MÉTODO: a través de la metodología ADAPTE un grupo de desarrollo de la guía, integrado por múltiples especialistas que tratan pacientes con rinitis alérgica, valoró la calidad de la guía ARIA 2010, revisó y tradujo las preguntas clínicas clave y ajustó las recomendaciones, preferencias del paciente y comentarios a la realidad mexicana (seguridad, costos y aspectos culturales). Para lograrlo se corrieron páneles Delphi, con tantas rondas como fuera necesario hasta lograr un acuerdo. Por medio de una revisión sistemática de la bibliografía se creó una pregunta especial no incluida en ARIA 2010 de la utilidad de realizar lavados nasales en pacientes con rinitis alérgica. RESULTADOS: se incluyeron 45 preguntas de la guía original ARIA 2010, divididas en seis bloques que abarcan prevención, tratamiento médico, inmunoterapia y terapias alternativas de pacientes con rinitis alérgica con o sin asma. La mayor parte de las preguntas alcanzaron acuerdo en una a dos rondas, sólo una requirió tres para ello. CONCLUSIONES: se cuenta ahora con una guía de rinitis alérgica de usosencillo, adaptada, actualizada y válida para México.

[Mexican guidelines on the diagnosis and treatment of urticaria]. / Guía Mexicana para el Diagnóstico y el Tratamiento de la Urticaria.

BACKGROUND: Urticaria is a disease that a fifth of the population shallsuffer once in a lifetime. Recent clinical guidelines have proposed some fundamental changes in the diagnosis and treatment of urticaria, making the development of a national, multidisciplinary guideline, with wide acceptability among different professional groups -both specialists and primary health care workers-, necessary in Mexico. MATERIAL AND METHOD: Internationally recognized tools for guidelinedevelopment were used. An interdisciplinary group of clinical experts (some of them knowledgeable in methodology of guideline development) determined the objectives and scope of the Evidence Based Clinical Practice Guideline with SCOPE. It was decided to adapt and transculturize international guidelines on the diagnosis and treatment of urticaria. With AGREE-II three high-quality guidelines (Zuberbier 2014, Sánchez-Borges 2012, Powell 2007) were selected to function as basic guidelines (BG). A set of Clinical Questions was formulated that lead to recommendations/suggestions, based on these BG, taking into account the cultural and economic background of Mexico, according to GRADE recommendation development. RESULTS: By a formal process of discussion and voting during several working-sessions, experts and first level healthcare physicians determined the wording of the final guideline, taking particularly care of developing a document, adjusted to the reality, values and preferences of the Mexican patients. The use of oral second generation, non-sedating antihistamines as first line treatment is emphasized. CONCLUSION: This document is an Evidence Based Clinical Practice Guideline for the diagnosis and treatment of acute and chronic urticaria, based on three, high quality, international guidelines. It was developed by a multidisciplinary group. Tables and algorithms make the guideline user-friendly for both, first line health care physicians and specialists.

Antecedentes: la urticaria es una enfermedad que padece una quinta parte de la población en algún momento de su vida. Las guías internacionales recientes han propuesto unos cambios de fondo en su diagnóstico y tratamiento, por lo que había la necesidad de crear una guía nacional y multidisciplinaria, con base amplia en los gremios de especialistas y médicos de primer contacto en México. Material y método: un grupo interdisciplinario de expertos clínicos y algunos expertos en metodología determinó los objetivos y alcances de la Guía de Práctica Clínica Basada en Evidencia con el instrumento SCOPE. Se decidió llevar a cabo la adaptación y transculturización de guías internacionales para el diagnóstico y tratamiento de urticaria. Con el instrumento AGREE-II se seleccionaron las tres guías de alta calidad, como guías base (Zuberbier 2014, Sánchez-Borges 2012, Powell 2007) para formular y contestar la preguntas clínicas clave, en el contexto cultural y económico mexicano, según el método de desarrollo de recomendaciones GRADE. Resultados: mediante un proceso formal de discusión y votación durante varias juntas de expertos, se terminó la redacción de la forma final de la guía, con especial cuidado de lograr un ajuste a las realidades, valores y preferencias de los pacientes de México. Se hace hincapié en la administración de antihistamínicos vía oral de segunda generación, como tratamiento de primera elección. Conclusión: este documento es una Guía de Práctica Clínica Basada en Evidencia para el diagnóstico y tratamiento de urticaria aguda y crónica, basada en tres guías internacionales de alta calidad. Se desarrolló por un grupo multidisciplinario. Los cuadros y algoritmos hacen a la guía amigable para su uso por médicos de primer contacto y por especialistas.

[Immunological therapy in asthma]. / Terapia inmunológica en asma.

The goals of pharmacological treatment of asthma is to achieve clinical control and prevent exacerbations with the minimal adverse effects. Pharmacologic therapies are categorized in two general classes: long-term and quick-relief medications. Local or systemic corticosteroids blockade the late phase reaction of the immunological inflammatory response and reduce airway hyperresponsiveness. This reaction can also be diminished by allergen specific immunotherapy, this treatment also create immunological tolerance for the allergen and prevents new sensitizations. Immunosuppressants and immunomodulators such as methotrexate can be used as part of therapy in patients who does not respond to the recommended treatment, these medications should be used only selected patients under the supervision of an asthma specialist, as their potential beneficial effect may not outweigh the risk of serious side effects. Biomolecular therapy medications, such as etarnecept, are not recommended, more studies are required. The anti-IgE monoclonal antibody, omalizumab, is recommended in adults and children over 12, who have allergy as an important cause of their asthma. In reference to childhood immunizations, there is no evidence of influence on the incidence of asthma, possible beneficial effect on asthma exacerbations with anti-influenzae vaccine. Finally we'll see some interesting points about pharmacogenetics.

[Autoimmune urticaria. Treatment with methotrexate]. / Urticaria autoinmunitaria. Tratamiento con metotrexato.

BACKGROUND: It has been established that 27-50% of patients with idiopathic chronic urticaria have antibodies directed against the alpha chain of the high-affinity IgE receptor, which are indirectly detected by cutaneous tests with autoserum. Thus, an autoimmune urticaria diagnosis can be settled. OBJECTIVE: To prove methotrexate's efficiency in patients with autoimmune urticaria. MATERIAL AND METHODS: Seven patients took part in the study. A series of tests was performed in order to rule out any possible infectious, metabolic, or physical etiology. Initial treatment with methotrexate with doses of 2.5 mg every 12 hours, two days a week was provided. In case there were no toxicity data, doses would increase to three days a week for a 6-week period. RESULTS: Statistically significant improvement was observed in the itching, as well as the presence of spots, repercussion on daily activities, sleep disorders. There was no statistical difference regarding the extension of the lesions and the presence of angioedema. Adverse effects were not significant. CONCLUSION: We conclude that methotrexate is effective in the treatment of autoimmune urticaria.

[Nasal remodeling in patient with perennial allergic rhinitis]. / Remodelación nasal en pacientes con rinitis perenne alérgica.

BACKGROUND: Remodeling is considered a new or different reconstruction of tissues. The airway remodeling is a consequence of changes induced by endothelial cells and extra-cellular matrix as a result of chronic inflammation. OBJECTIVE: To show histological changes into mucosa in patients with perennial allergic rhinitis. MATERIAL AND METHODS: We studied 26 patients with perennial allergic rhinitis, with evolution of 3 to 6 years, between 20 to 40 years old, without medical or immunological treatment. A turbinate biopsy was taken in all of them. RESULTS: We found enlargement of the basal membrane in 92.3%, subepithelial fibrosis in 92.4%, neutrophils in 100% of the cases, eosinophils in 100%, lymphocytes in 100%, edema in 46.2%, fragmented reticular fibers in 19.2%, and vascular dilatation in 11.1%. CONCLUSION: These results suggest nasal remodeling in patients with allergic rhinitis, although more structural studies about this pathology are necessary.

Hallazgos radiológicos en pacientes con rinitis alérgica perenne / Radiological findings in patients with perennial allergic rhinitis

Antecedentes: la rinitis se define clínicamente por congestión, estornudos, prurito y rinorrea. Material y método: estudio prospectivo y longitudinal efectuado en 69 pacientes consecutivos del servicio de inmunología clínica y alergia del Hospital de Especialidades del CMN Siglo XXI. Resultados: del total de pacientes 53 eran mujeres y 16 hombres, con límites de edad entre 15 y 55 años, con diagnóstico de rinitis alérgica perenne. Todos los sujetos de estudio tuvieron antecedentes de atopia; 50.7 por ciento asma con enfermedad agregada, 47.8 por ciento poliposis nasal, 27.5 por ciento sinusitis, 59.9 por ciento en el maxilar, 36.2 por ciento etmoidal y frontal en 7.2 por ciento. Con desviación septal se encontró al 33.3 por ciento de los pacientes, 7.2 por ciento con desviación septal más espolen, obstrucción del complejo osteomeatal 4.3 por ciento, hipertrofia de cornetes 53.5 por ciento, pólipos 27.5 por ciento y concha bulosa 7.2 por ciento. El diagnóstico se realizó con tomografía axial de senos paranasales. La chi al cuadrado determinó la asociación entre las alteraciones estructurales y la sinusitis con una p = 0.001, con estrecha relación entre rinitis alérgica y sinusitis. Conclusiones: se demostró la importancia del diagnóstico y tratamiento oportuno de esta afección.

Resultados de un programa educativo para adultos asmáticos / Result of an educational program for asthmatic adults

El asma bronquial es una enfermedad crónica, que representa un enorme costo económico y social. Los programas educativos dirigidos a pacientes asmáticos disminuyen la morbilidad y mejoran la calidad de vida de los pacientes, con menor costo de la atención médica. Se muestran los resultados de un programa educativo para adultos asmáticos que fue evaluado duarante un año después de su implantación, a través de encuestas. Los resultados muestran disminución en el número de los medicamentos empleados, en él numero de crisis asmátcas, en los días de incapacidad laboral y ausentismo escolar, así como en el número de consultas en servicios de urgencias y hospitales generales de zona.