Clinical Practice Guidelines: Screening, Diagnosis and Management of Acute Events and Prophylaxis of Hereditary Angioedema (HAE)

El AEH es una patología genética, enfermedad rara, con una prevalencia aproximada entre 1 por cada 50,000 habitantes, caracterizado por episodios de edemas a nivel subcutáneo y de mucosas (abdominal, genitourinario, respiratoria), siendo potencialmente mortal cuando hay afectación de la vía aérea superior. En Perú se estiman 600 pacientes con AEH. El AEH se puede clasificar: con alteración del C1 inhibidor (Tipo I y II), y sin alteración del C1 inhibidor (denominado anteriormente Tipo III). El diagnóstico de laboratorio: C4 complemento, C1 inhibidor esterasa cuantitativo y cualitativo, estudios genéticos. Existen tratamientos específicos a nivel mundial para crisis agudas y profilaxis en AEH, en Perú, el único tratamiento registrado actualmente es Ecallantide, útil en crisis agudas, además podemos utilizar tratamientos alternativos como el ácido tranexámico y el danazol. Presentamos la primera Guía de Práctica Clínica en Perú para el Tamizaje, Diagnóstico y Manejo de Episodios Agudos y Profilaxis en pacientes AEH.HAE is a genetic pathology, a rare disease with a prevalence of approximately 1 per 50,000 inhabitants, characterized by episodes of edema at the subcutaneous level and mucous membranes (abdominal, genitourinary, respiratory), being potentially fatal when there is involvement of the glottis. In Peru, there are an estimated 600 patients with HAE. HAE can be classified as follows: with C1 inhibitor alteration (Type I and II), and without C1 inhibitor alteration (previously called Type III). Laboratory diagnosis: C4 complement, C1 inhibitor esterase quantitative and qualitative, genetic studies. There are specific treatments worldwide for acute crises and prophylaxis in HAE; in Peru the only treatment currently registered is Ecallantide, useful in acute crises; we can also use alternative treatments such as tranexamic acid and danazol. We present the first Clinical Practice Guide in Peru for Screening, Diagnosis and Management of Acute Episodes and Prophylaxis in HAE Patients

Algoritmo de tratamiento de la dermatitis atópica en Perú. Consenso de expertos

Introducción: La dermatitis atópica es una enfermedad crónica, sistémica, reincidente, con manifestaciones dermatológicas, que impone una alta carga a los pacientes, las familias y los sistemas de salud, y tiene repercusión psicológica, social y económica, y en la calidad de vida de los pacientes. Afecta principalmente a la población pediátrica y con menor frecuencia a la adulta. Las manifestaciones clínicas varían según la edad y evolución de la enfermedad, y en la actualidad se dispone de múltiples opciones farmacológicas y no farmacológicas para el tratamiento de los pacientes. Objetivo: Presentar un algoritmo de tratamiento de la dermatitis atópica, propuesto con una serie de recomendaciones acerca del diagnóstico, tratamiento y seguimiento de los pacientes. Métodos: Se llevó a cabo un consenso de expertos, utilizando la técnica del grupo nominal. La metodología se desarrolló en 7 fases que incluyeron: planteamiento de las preguntas de investigación, búsqueda de la bibliografía, propuesta inicial de las recomendaciones, elaboración de las recomendaciones finales y del algoritmo de tratamiento con tres ciclos de votación. Se estableció el consenso con un 80% de favorabilidad. Resultados y conclusiones: El resultado del consenso fue un algoritmo de tratamiento de pacientes con dermatitis atópica leve, moderada-grave, derivado de las recomendaciones de expertos. En el algoritmo se establecen criterios diagnósticos y de tratamiento, y se aportan recomendaciones actualizadas que incluyen las alternativas disponibles en Perú

Learnings from real-life experience of using omalizumab for chronic urticaria in Latin America

Background: Updated urticaria guidelines recommend that patients should be assessed for disease activity, severity, control, and quality of life at baseline and follow up. Regarding treatment, guidelines consider second generation antihistamines as the cornerstone in therapy for chronic urticaria (CU), while other drugs, such as omalizumab, are conceived as second-line alternatives. In regards to omalizumab, despite advances in the management of CU, there are still open questions about timing, dosing, and objective measures for clinical response. This study was designed to portray the use of patient-reported outcomes (PROs) in chronic urticaria management, as well as the effectiveness and treatment patterns of omalizumab in CU, as seen in a real-life setting in Latin America. Methods: This is a retrospective observational study, involving 72 Latin American patients with chronic urticaria treated with omalizumab. Patient reported outcomes and treatment patterns, response, quality of life improvement and discontinuation were analyzed. Results: From the 72 patients, 91.7% (n = 66) were assessed through PROs, where urticaria control test (UCT) was the most used (79.2%; n = 57). Overall, 80.0% (n = 44) responded to omalizumab at some point of the treatment. Omalizumab 300 mg was associated with earlier response compared to lower doses. Regardless of dosage, most patients assessed with CU-Q2oL improved quality of life (80.8%; n = 21). With respect to omalizumab discontinuation, 20.8% (n = 15) patients interrupted omalizumab before the 3rd month of treatment (p = .000). Conclusions: The present study highlights how the use of PROs and omalizumab in Latin America differ from guidelines’ recommendations and clinical trials. Even though most patients were initiated under omalizumab 300 mg, most of them finished with lower doses. Regardless of dosage, most patients responded to omalizumab and improved quality of life at some point during treatment. However, such features were seen earlier with omalizumab 300 mg. Regarding treatment discontinuation, one-fifth of patients interrupted omalizumab before the third month. Keywords: Chronic spontaneous urticaria, Patient-reported outcomes, Omalizumab, Quality of life, Latin Americ

The global impact of the COVID-19 pandemic on the management and course of chronic urticaria

Introduction: The COVID-19 pandemic dramatically disrupts health care around the globe. The impact of the pandemic on chronic urticaria (CU) and its management are largely unknown. Aim: To understand how CU patients are affected by the COVID-19 pandemic; how specialists alter CU patient management; and the course of CU in patients with COVID-19. Materials and Methods: Our cross-sectional, international, questionnaire-based, multicenter UCARE COVID-CU study assessed the impact of the pandemic on patient consultations, remote treatment, changes in medications, and clinical consequences. Results: The COVID-19 pandemic severely impairs CU patient care, with less than 50% of the weekly numbers of patients treated as compared to before the pandemic. Reduced patient referrals and clinic hours were the major reasons. Almost half of responding UCARE physicians were involved in COVID-19 patient care, which negatively impacted on the care of urticaria patients. The rate of face-to-face consultations decreased by 62%, from 90% to less than half, whereas the rate of remote consultations increased by more than 600%, from one in 10 to more than two thirds. Cyclosporine and systemic corticosteroids, but not antihistamines or omalizumab, are used less during the pandemic. CU does not affect the course of COVID-19, but COVID-19 results in CU exacerbation in one of three patients, with higher rates in patients with severe COVID-19. Conclusions: The COVID-19 pandemic brings major changes and challenges for CU patients and their physicians. The long-term consequences of these changes, especially the increased use of remote consultations, require careful evaluation

The global impact of the COVID-19 pandemic on the management and course of chronic urticaria

Introduction: The COVID-19 pandemic dramatically disrupts health care around the globe. The impact of the pandemic on chronic urticaria (CU) and its management are largely unknown. Aim: To understand how CU patients are affected by the COVID-19 pandemic; how specialists alter CU patient management; and the course of CU in patients with COVID-19. Materials and methods: Our cross-sectional, international, questionnaire-based, multicenter UCARE COVID-CU study assessed the impact of the pandemic on patient consultations, remote treatment, changes in medications, and clinical consequences. Results: The COVID-19 pandemic severely impairs CU patient care, with less than 50% of the weekly numbers of patients treated as compared to before the pandemic. Reduced patient referrals and clinic hours were the major reasons. Almost half of responding UCARE physicians were involved in COVID-19 patient care, which negatively impacted on the care of urticaria patients. The rate of face-to-face consultations decreased by 62%, from 90% to less than half, whereas the rate of remote consultations increased by more than 600%, from one in 10 to more than two thirds. Cyclosporine and systemic corticosteroids, but not antihistamines or omalizumab, are used less during the pandemic. CU does not affect the course of COVID-19, but COVID-19 results in CU exacerbation in one of three patients, with higher rates in patients with severe COVID-19. Conclusions: The COVID-19 pandemic brings major changes and challenges for CU patients and their physicians. The long-term consequences of these changes, especially the increased use of remote consultations, require careful evaluation

Definition, aims, and implementation of GA 2 LEN/HAEi angioedema centers of reference and excellence

This document summarizes the aims of GA2 LEN/HAEi Angioedema Centers of Reference and Excellence (ACAREs) and elaborates the requirements that ACAREs must fulfill to become certified. It also provides (see Appendix S1) background information on GA2LEN and HAEi, including HAEi member organizations and regional patient advocates, on why we need an Angioedema Center of Reference and Excellence (ACARE) program and network, and on the accreditation and certification process, governance and funding, and on the interaction with other GA2LEN networks of centers of reference and excellence. The protocols, aims, requirements, and provisions related to becoming a certified CARE are based on (a) the experience of the GA2LEN UCARE network and (b) input from angioedema patients, general practitioners, and angioedema specialists