Cold-induced anaphylaxis triggered by drinking cold water

Cold urticaria is an inducible urticaria in which hives and angioedema appear after exposure to cold. The symptoms of cold urticaria often are limited to hives/angioedema. However, in up to 20% of cases, cold exposure may trigger anaphylaxis. We report the case of an 11-year-old boy previously diagnosed with chronic spontaneous urticaria who developed facial swelling, itchy hives, difficulty in breathing, vomiting and abdominal pain within 5 minutes of drinking cold water. He received a standard dose of non-sedating second-generation antihistamines at home. He was observed in the emergency room for 2 hours and discharged with an epinephrin autoinjector. During the subsequent outpatient clinic visit, an ice cube test was performed which confirmed the new diagnosis of comorbid cold-induced chronic urticaria. On further questioning, the parents reported occurrence of hives following swimming in the swimming pool. Cold-induced urticaria should be suspected in cases of anaphylaxis associated with cold exposure. Patients with chronic forms of urticaria who present with new anaphylaxis should be assessed for a potential concomitant cold-induced form. (AU)

Angioedema postoperatorio de inicio tardío, desencadenado por un inhibidor de la enzima convertidora de angiotensina: previsión de vía aérea de emergencia / Late-onset postoperative angioedema triggered by angiotensin-converting enzyme inhibitor: an emergent airway forethought

El angioedema es una situación potencialmente mortal, debido al riesgo de compromiso de la vía aérea que da lugar a un deterioro de la función respiratoria, hipoxia y, por último, paro cardiopulmonar. Puede ser provocado o desencadenado por agentes farmacéuticos, factores emocionales o fisiológicos, traumatismo de la vía aérea superior, o estrés quirúrgico.Un varón de 46 años de edad, a quien se había prescrito previamente perindopril, desarrolló angioedema de lengua 4h después de recibir el alta de la UCPA (unidad de cuidados postanestésicos). Se convocó a un equipo multidisciplinar, que destacó una estrategia de manejo de la vía aérea en caso de empeoramiento. Dicha estrategia consistió en intubación con fibroscopio por parte del anestesiólogo, o bien traqueostomía quirúrgica practicada por el equipo de cirugía, realizándose ambas técnicas con el paciente despierto y ventilación espontánea.El objetivo de este informe de caso es alertar de que el angioedema es una situación potencialmente mortal. Para un manejo óptimo, es importante preparar de antemano una estrategia detallada de manejo de la vía aérea, a implementar por parte de un equipo multidisciplinar. (AU)

Angioedema is a potentially life-threatening condition due to the risk of airway compromise leading to deterioration of respiratory function, hypoxia, and ultimately, cardiopulmonary arrest. It can be either unprovoked or triggered by pharmaceutical agents, emotional or physiologic factors, upper airway trauma, or surgical stress.A 46-year-old man previously prescribed perindopril developed angioedema of the tongue 4h after being discharged from the Post Anesthesia Care Unit (PACU). A multidisciplinary team was called and they outlined an airway management strategy to use in the event of worsening. The strategy consisted of either fiberoptic intubation by an anesthesiologist or surgical tracheostomy performed by the surgical team, both performed with the patient awake and in spontaneous ventilation.The aim of this case report is to raise awareness that angioedema is a potentially life-threatening condition. For optimal management, it is important to prepare in advance a detailed airway management strategy to be implemented by a multidisciplinary team. (AU)

An unusual case of acquired angioedema associated with monoclonal gammopathies of uncertain significance

Acquired angioedema (AAE) is a rare disease due to the C1 esterase inhibitor (C1-INH) deficiency. Clinically, its symptoms are similar to hereditary angioedema (HAE) with hereditary C1-INH deficiency. Both conditions have the potential to cause upper airway obstruction, which can be fatal in clinical practice and thus require intense attention. Here, we’d like to discuss the clinical presentation, diagnosis and follow up of a special case of AAE associated with monoclonal gammopathies of unknown significance (MGUS) with recurrent upper airway obstruction. The patient was regularly followed up after being discharged from our ward. Measurements of C3–C4 levels were carried out by a hematological test. Due to the rarity of such a disease, especially in Chinese people, relevant diagnosis methods are missing in this patient, so the patient was only diagnosed with AAE-C1-INH associated with MGUS clinically. The latest follow up showed that he still underwent recurrent upper airway obstruction; thus, he remained in a tracheostomy state due to a lack of proper medication prophylaxis and died eventually. This unusual case reminds emergency physicians to pay attention to such disease during clinical practice, and relevant diagnosis method should be improved (AU)

A new classification option for NSAID hypersensitivity: Kalyoncu classification

Background The European Network for Drug Allergy (ENDA) proposed a consensus document for hypersensitivity reactions to nonsteroidal anti-inflammatory drugs (NSAIDs) in 2011. A subgroup of patients with NSAIDs-exacerbated respiratory disease (NERD) develop urticaria/angioedema type reactions in response to NSAIDs. The Kalyoncu classification might be a novel option to classify patients with NSAID hypersensitivity (NH). In this study, we compare the ENDA and the Kalyoncu classifications. Methods This study enrolled a total of 196 patients. NH reaction types were categorized as asthma, rhinitis, urticaria/angioedema and anaphylaxis. Based on the reaction history and oral provocation test findings, patients were grouped according to ENDA and Kalyoncu classifications. Results The mean age of the 196 patients was 40.32±13.28 years, and 130 (66.3%) of them were female. Under the ENDA and Kalyoncu classifications, the most common NH subgroups were NERD (32%), and isolated NH (34.2%), the least prevalent NH subgroups were single NSAID-induced delayed reactions (SNIDR) (1.5%), and pseudo Samter’s syndrome (11.7%). Conclusions Our research revealed that the Kalyoncu classification is more descriptive of patients with NERD exhibiting urticaria/angioedema-type reactions. It also provides future risk assessment for development of NERD. For controversial cases, the Kalyoncu classification can be utilized as a new complimentary option alone or in conjunction with ENDA classification (AU)

Etiological causes in patients with acute urticaria and angioedema: A cross-sectional study

Background: Acute urticaria and angioedema are emergency dermatological conditions associated with various etiologic factors. Objective: To determine the etiological causes in patients with acute urticaria and angioedema, and to investigate whether more than one etiological cause was present, along with the patients’ laboratory values. Methods: The study was conducted in a tertiary hospital with one center. Etiological causes and laboratory parameters in 284 patients diagnosed with acute urticaria and angioedema were retrospectively studied. Results: A total of 284 patients were included in the study. The mean age of the patients was 42.7 ± 15.6 years, where 163 (57.4%) were women and 121 (42.6%) were men. Acute urticaria and angioedema occurred together in 149 (52.5%) patients. At least one precipitating factor among the predisposing risk factors was present in 220 (77.5%) patients, and more than one precipitating factor was found in 51 (18%) patients. Medication use was found in 157 (55.3%) patients and infection in 54 (19%). The development of urticaria after food consumption was noted in nine (3.2%) individuals. A history of infection and medication intake was present in 50 (17.6%) patients. A joint history of food consumption and medication intake was present in only one patient. Elevated C-reactive protein level was found in 178 (62.7%) patients and elevated anti-streptolysin O titer in 41 (14.4%) patients. Vitamin B12 deficiency was found in 116 (40.8%) patients and vitamin D deficiency in 254 (89.4%). Conclusion: Acute urticaria and angioedema may occur as a result of multiple etiological factors, in which different triggers may be present simultaneously (AU)

Urticaria and angioedema in children and adolescents: diagnostic challenge

Urticaria diagnosis may be challenging in children since it can be triggered or related to numerous conditions. In this paper, we reviewed the main aspects regarding the diagnosis of urticaria in the pediatric population. Acute urticaria is often due to viral infections. However, other culprits, including foods, insect stings, drugs, contrast media, vaccination, latex, and medical diseases, may account for acute patterns. Laboratory tests and confirmatory allergy tests should be individualized and guided by history. Chronic urticaria (CU) is defined when hives and/or angioedema last for more than 6 weeks. The most common type of chronic urticaria in children is chronic spontaneous urticaria (CSU). Chronic inducible urticaria (CindU) is less common but is important to diagnose in order to manage appropriately and reduce the risk of severe reactions. Inducible forms in children are often diagnosed with specific provocation tests similar to the tests used in adults. Given that chronic urticaria could rarely be a presentation of vasculitis, systemic-onset juvenile idiopathic arthritis, or auto-inflammatory syndromes, it is important to rule out these conditions. It is crucial to differentiate cases of chronic urticaria from mastocytosis and Bradykinin-mediated angioedema, given that treatment may differ. The management of chronic urticaria in children has improved over the last decade because of the development of both clear management guidelines and new effective drugs. It is crucial to increase awareness for appropriate diagnosis and new available treatment to improve the management of chronic urticaria in children (AU)

A Specialized Therapeutic Approach to Chronic Urticaria Refractory to H1-Antihistamines Improves Disease Burden: The Spanish AWARE Experience

Objective: During its first year, the AWARE study assessed disease activity, patient quality of life (QOL), and treatment patterns in chronic urticaria (CU) refractory to H1-antihistamines (H1-AH) in clinical practice.Methods: We performed an observational, prospective (24 months), international, multicenter study. The inclusion criteria were age ≥18 years and H1-AH–refractory CU (>2 months). At each visit, patients completed questionnaires to assess disease burden (Urticaria Control Test [UCT]), disease activity (7 day-Urticaria Activity Score [UAS7]), and QOL (Dermatology Life Quality index [DLQI], Chronic Urticaria Quality of Life Questionnaire [CU-Q2oL], and Angioedema Quality of Life Questionnaire [AE-QoL]). We present data for Spain.Results: The study population comprised 270 evaluable patients (73.3% female, mean [SD] age, 48.9 [14.7] years). At baseline, 89.3% were prescribed a CU treatment. After 1 year, first- and second-line treatments became less frequent and third-line treatments became more frequent. At baseline, 47.0% of patients experienced angioedema; at 1 year, this percentage had fallen to 11.8%. The mean (SD) AE-QoL score decreased from 45.2 (28.7) to 24.0 (25.8). The mean (SD) UCT score decreased from 7.0 (4.5) to 12.1 (4.1). According to UAS7, 38.2% of patients reported absence of wheals and itch in the previous 7 days at 1 year compared with 8.3% at baseline. The mean (SD) DLQI score decreased from 8.0 (7.4) to 2.8 (4.6). At the 1-year visit, the percentage of patients reporting a high or very high impact on QOL fell from 29.9% to 9.6%.Conclusions: H1-AH–refractory CU in Spain is characterized by absence of symptoms and a considerable impact on QOL. Continuous follow-up of CU patients and third-line therapies reduce disease burden and improve patients’ QOL (AU)

Objetivo: El estudio AWARE evalúa la actividad de la enfermedad, la calidad de vida (CV) del paciente y los patrones de tratamientoen pacientes con urticaria crónica (UC) refractarios a antihistamínicos H1 (AH-H1) en práctica clínica durante el primer año del estudio.Métodos: Estudio observacional, prospectivo (24 meses), internacional y multicéntrico. Pacientes ≥18 años con diagnóstico de UC refractariosa AH-H1 (>2 meses). En cada visita, los pacientes completaron cuestionarios para evaluar la carga de la enfermedad (Urticaria Control Test[UCT]), actividad de la enfermedad (7 day-Urticaria Activity Score [UAS7]), CV (Dermatology Life Quality index [DLQI], Chronic UrticariaQuality of Life Questionnaire [CU-Q2oL], Angioedema Quality of Life [AE-QOL]). Presentamos datos españoles.Resultados: Se incluyeron 270 pacientes evaluables (73,3% mujeres, edad media [DE] 48,9 [14,7] años). Al inicio del estudio, al 89,3%se le prescribió un tratamiento para la UC. Después de 1 año, los tratamientos de primera/segunda línea tendieron a disminuir y la tercera línea a aumentar. El 47,0% de los pacientes experimentaron angioedema al inicio del estudio, siendo del 11,8% al cabo de 1 año. Lamedia (DE) de AE-QOL pasó de 45,2 (28,7) a 24,0 (25,8). La media (DE) de UCT pasó de 7,0 (4,5) a 12,1 (4,1). Según UAS7, el 38,2% depacientes reportaron ausencia de ronchas y prurito en los últimos 7 días al año frente al 8,3% al inicio. El DLQI medio (DE) pasó de 8,0 (7,4)a 2,8 (4,6). En la visita de 1 año, el porcentaje de pacientes que reportaron un impacto en la CV alto/muy alto pasó del 29,9% al 9,6%.Conclusiones: Los pacientes españoles con UC refractarios a AH-H1 presentan una falta de control de la sintomatología con un importanteimpacto en su CV. El seguimiento continuo de los pacientes con urticaria crónica espontánea y las terapias de tercera línea han demostradouna tendencia a reducir la carga de la enfermedad y a mejorar la CV de los pacientes (AU)

Manejo del angiodema inducido por inhibidores de la enzima convertidora de angiotensina con icatibant / Management of angiotensin-converting enzyme inhibitors-related angioedema with icatibant

Objetivo: Evaluar la efectividad de icatibant en el tratamiento del angioedema inducido por IECA (AII), en un centro sin protocolización previa del manejo.Método: Estudio observacional retrospectivo y descriptivo. Se incluyeron pacientes diagnosticados de AII y tratados con icatibant 30 mg entre mayo 2015-diciembre 2017. Las variables de resultado principal y secundaria fueron: tiempo hasta resolución completa y tiempo hasta primera mejoría; respectivamente.Resultados: Cinco pacientes, mediana de edad 76 años (46-81); cuatro mujeres y un varón. Todos caucásicos. Medianas de tiempo hasta resolución completa y hasta primera mejoría: 23 horas (IQR 20,0-25,0) y 3 horas (IQR 3,0-6,0); respectivamente.Conclusiones. El inicio temprano del tratamiento anti-bradicinérgico puede resultar clave para la evolución del cuadro. Para alcanzar la máxima efectividad, se reduzcan las morbilidades asociadas, los ingresos en UCI y el tiempo de estancia hospitalaria, resulta primordial la elaboración de protocolos locales que tengan en cuenta las particularidades de cada centro. (AU)

Objetive: To assess the effectiveness of icatibant in the management of angiotensin-converting enzyme inhibitor-induced angioedema (AII) in a hospital without a treatment guidance.Methods: Observational, retrospective and descriptive study. All patients diagnossed with AII and treated with icatibant 30 mg between May 2015-December 2017 were included. The primary and secondary end-points were: time to total resolution and time to first improvement; respectively.Results: Five patients, median age 76 years (46-81). Four women and a man. All of them Caucasian. Median time to total resolution and to first improvement: 23 hours (IQR 20.0-25.0) and 3 hours (IQR 3.0-6.0); respectively.Conclusion: The early start with the anti-bradicinergic therapy may be key to the AII evolution. To achieve the maximum effectiveness and to get reduced the associated morbidity, the ICU admission and the time to discharge, the development of local protocols considering the particularities of each center is highly necessary. (AU)