Delayed postoperative reactions to metamizole: a diagnostic challenge.

Summary: Background. Metamizole, a non-steroidal anti-inflammatory drug from the pyrazolone group, is a frequent cause of immediate hypersensitivity reactions and, more rarely, of delayed drug hypersensitivity reactions. Due to its favorable pharmacokinetic characteristics, metamizole is widely used in the postoperative period for pain control. Methods. Retrospective study of patients referred for allergological study between January 2012 and June2022 for postoperative hypersensitivity reactions. Clinical and diagnostic data were collected through review of patients' medical records. Twenty patients with postoperative hypersensitivity reactions were referred, of which 10 presented delayed reactions. We analyzed the results of skin prick, intradermal and patch tests performed with an intravenous metamizole solution as well as provocation tests performed with metamizole and acetylsalicylic acid. Cross-reactivity to non-steroidal anti-inflammatory drugs was excluded by confirmation of clinical tolerance to non-steroidal anti-inflammatory drugs or by acetylsalicylic acid provocation test. Results. In 7 of the 10 patients a delayed reaction to metamizole was diagnosed. These reactions were characterized as maculopapular exanthema, occurring in multiple postoperative settings. Skin tests were negative, except in one patient with late mild erythema in the ipsilateral upper limb and no reaction at the site of intradermal injection. Delayed hypersensitivity was demonstrated by late positive metamizole provocation tests. Conclusions. This study demonstrated that for a correct diagnosis a high degree of suspicion about possible delayed hypersensitivity drug reactions to metamizole in the postoperative setting is needed. In the investigation, provocation test with metamizole was decisive for diagnostic confirmation.

Evolution of Guidelines for the Management of Hereditary Angioedema due to C1 Inhibitor Deficiency.

Hereditary angioedema (HAE) is a severe and disabling condition characterized by recurrent episodes of subcutaneous or mucosal swelling in the skin and respiratory and gastrointestinal tracts. HAE due to C1-esterase inhibitor deficiency (C1-INH-HAE) is the most prevalent subtype. The present Iberian study compared C1-INH-HAE treatment guidelines published between 2010 and 2022 to identify the main differences in therapeutic approaches for on-demand treatment and short- and long-term prophylaxis (LTP). HAE guidelines evolved with the availability of new treatments and with a change in the management paradigm towards an individualized, patient-centered approach, where quality of life (QOL) is central. A parallel trend was observed towards increasingly frequent home-based treatment, which potentially facilitates timely interventions, provides greater flexibility and convenience, and is associated with increased QOL, enabling patients to lead more normal lives. Most innovations over the years were made for LTP, together with the advent of new therapies and awareness of patients' needs. Several prophylactic therapies with a high level of evidence became available, although formal head-to-head comparisons are lacking. The treatment goals became more ambitious, ranging from a reduction in the frequency, severity, and duration of attacks to achieving total disease control and normalization of patients' lives. The document also addresses relevant items such as changes in terminology (eg, the introduction of designations as "first-line") and the introduction of patient-reported outcome measures to assess patients' perceptions of their self-experienced QOL and well-being. Unmet needs in the management of C1-INH-HAE are identified.

Evolution of Guidelines for the Management of Hereditary Angioedema due to C1 Inhibitor Deficiency

Hereditary angioedema (HAE) is a severe and disabling condition characterized by recurrent episodes of subcutaneous or mucosal swelling in the skin and respiratory and gastrointestinal tracts. HAE due to C1-esterase inhibitor deficiency (C1-INH-HAE) is the most prevalent subtype. The present Iberian study compared C1-INH-HAE treatment guidelines published between 2010 and 2022 to identify the main differences in therapeutic approaches for on-demand treatment and short- and long-term prophylaxis (LTP). HAE guidelines evolved with the availability of new treatments and with a change in the management paradigm towards an individualized, patient-centered approach, where quality of life (QOL) is central. A parallel trend was observed towards increasingly frequent home-based treatment, which potentially facilitates timely interventions, provides greater flexibility and convenience, and is associated with increased QOL, enabling patients to lead more normal lives. Most innovations over the years were made for LTP, together with the advent of new therapies and awareness of patients’ needs. Several prophylactic therapies with a high level of evidence became available, although formal head-to-head comparisons are lacking. The treatment goals became more ambitious, ranging from a reduction in the frequency, severity, and duration of attacks to achieving total disease control and normalization of patients’ lives. The document also addresses relevant items such as changes in terminology (eg, the introduction of designations as “first-line”) and the introduction of patient-reported outcome measures to assess patients’ perceptions of their self-experienced QOL and well-being. Unmet needs in the management of C1-INH-HAE are identified (AU)

El angioedema hereditario (AEH) es una enfermedad grave e incapacitante, caracterizada por episodios recurrentes de edema subcutáneo en la piel o en las mucosas de los tractos respiratorio y gastrointestinal. El AEH por déficit del C1-inhibidor (AEH-C1-INH) es el subtipo más prevalente. En el presente estudio ibérico se han comparado las guías/recomendaciones de tratamiento del AEH-INH-C1, publicadas entre 2010 y 2022 para identificar las principales diferencias en cuanto a los enfoques terapéuticos para el tratamiento a demanda y la profilaxis a corto y largo plazo (PLP). A nivel mundial, las directrices sobre el AEH evolucionaron con la disponibilidad de nuevos tratamientos y con un cambio en el paradigma de gestión hacia un enfoque individualizado y centrado en el paciente en el que la calidad de vida (CdV) es fundamental. En consonancia con ello, se observó una tendencia creciente hacia el tratamiento domiciliario, ya que facilita potencialmente las intervenciones precoces, proporciona mayor flexibilidad y comodidad, y se asocia a una mayor calidad de vida, permitiendo a los pacientes llevar una vida normal. La PLP es el indicador que más innovaciones ha experimentado a lo largo de los años, paralelamente a la disponibilidad de nuevas terapias y a la toma de conciencia de las necesidades de los pacientes. Se dispone de varias terapias profilácticas con un alto nivel de evidencia, aunque faltan estudios específicos de comparaciones directas entre ellas. Los objetivos del tratamiento se han ido haciendo más ambiciosos, desde la reducción de la frecuencia, gravedad y duración de los ataques, hasta lograr el control total de la enfermedad y la normalización de la vida de los pacientes en la actualidad (AU)

Hereditary angioedema: 24 years of experience in a Portuguese Reference Center.

Summary: Hereditary angioedema (HAE) poses a high burden of disease, being its epidemiological and clinical data heterogeneous among countries, with no recent published studies concerning Portuguese patients. Therefore, we aimed to raise awareness of HAE and to contribute to clinical knowledge. An observational, descriptive, retrospective, and cross-sectional study was performed, that included a cohort of 126 patients followed in a single Portuguese Center. We observed a high prevalence of HAE-C1-INH type II (45.2% of patients). Most HAE patients (67.4%) presented the initial manifestations of the disease before adulthood, at a mean age of 12.6 ± 8.4 years. However, we found a long delay in HAE diagnosis, especially in those without family history (mean 20.7 ± 17.3 years). Stress was the most common trigger, followed by trauma and infection. Symptoms involving different systems were increasingly reported with increased disease duration. Cutaneous symptoms (95.0%) were more frequent, followed by gastrointestinal (80.7%), and respiratory symptoms (50.4%). HAE symptoms led to abdominal surgery in 22 (17.5%) patients and induced laryngeal edema requiring intubation/tracheostomy in 8 (6.3%) patients. Most patients were under long-term prophylaxis, mainly with attenuated androgens (62.7% of patients).The correct distinction between HAE and other common causes of angioedema is critical, allowing reduction of diagnostic delay, improvement of adequate management, and ultimately improving outcomes and quality of life of HAE patients.

Anaphylaxis: A Decade of a Nationwide Allergy Society Registry

Background: Anaphylaxis is an acute, life-threatening, multiorgan hypersensitivity reaction.Objective: The aim of this study was to identify the causes of anaphylaxis in Portugal in order to improve our knowledge of epidemiology and management.Methods: We implemented a nationwide notification system for anaphylaxis over a 10-year period, with voluntary reporting by allergists. Data on 1783 patients with anaphylaxis were included. Etiopathogenesis, manifestations, and clinical management were characterized in detail for both children and adults.Results: The mean age was 32.7 (20.3) years, and 30% were under 18 years of age; 58% were female. The mean age at the first anaphylaxis episode was 27.5 (20.4) years (ranging from 1 month to 88 years). The main culprits of anaphylaxis were foods (48%), drugs (37%) (main trigger in adults, 48%), and hymenoptera venom (7%). The main culprit foods were shellfish (27%), fresh fruit (17%), cow’s milk (16%), tree nuts (15%), fish (8%), egg (7%), and peanut (7%). The main drugs were nonsteroidal anti-inflammatory drugs (43%), antibiotics (39%), and anesthetic agents (6%). Other causes included exercise (3%), latex (2%), cold-induced anaphylaxis (2%), and idiopathic anaphylaxis (2%). Most patients (80%) were admitted to the emergency department; only 43% received adrenaline. Anaphylaxis recurred in 41% of patients (21% with ≥3 anaphylactic episodes); 7% used an adrenaline autoinjector device.Conclusions: Food is the leading cause of anaphylaxis in Portugal, while drugs were the main elicitors in adults. We emphasize undertreatment with adrenaline and recurrent episodes, highlighting the need to improve diagnostic and therapeutic approaches to anaphylaxis (AU)

Antecedentes: La anafilaxia es una reacción de hipersensibilidad sistémica potencialmente mortal.Objetivo: El objetivo de este estudio fue el identificar las causas de la anafilaxia en Portugal para mejorar el conocimiento de la epidemiologíay del manejo de la enfermedad.Métodos: Durante un período de 10 años, se implementó un sistema nacional de notificación de anafilaxia, por parte de los alergólogos,mediante la emisión de informes voluntarios. Se recogieron datos de 1.783 pacientes con anafilaxia, pediátricos y adultos, relativos a laetiopatogenia, las manifestaciones clínicas y el manejo clínico de la misma.Resultados: La edad media fue de 32,7 ± 20,3 años, siendo el 30% de los pacientes menores de 18 años. El 58% fueron mujeres. Laedad media del primer episodio de anafilaxia fue de 27,5 ± 20,4 años (desde 1 mes hasta 88 años). Los principales agentes responsablesde anafilaxia fueron los alimentos (48%), los medicamentos (37%), que fue el principal desencadenante en los pacientes adultos, y losvenenos de himenópteros (7%). Los principales alimentos responsables fueron: mariscos (27%), frutas frescas (17%), leche de vaca (16%),nueces (15%), pescado (8%), huevo (7%) y cacahuete (7%). Los principales fármacos, fueron los antiinflamatorios no esteroideos (43%),antibióticos (39%) y anestésicos (6%). Entre otras causas implicadas se incluyó: ejercicio físico (3%), látex (2%), frío (2%) e idiopática (2%). La mayoría de los pacientes fueron atendidos en el servicio de urgencias (80%), y solo el 43% recibió tratamiento con adrenalina.La recurrencia de la anafilaxia ocurrió en el 41% de los pacientes (21% con ≥3 episodios anafilácticos). El 7% utilizó un dispositivoautoinyector de adrenalina.Conclusiones: Los alimentos son la principal causa de anafilaxia en Portugal y en el subgrupo de pacientes adultos, lo son los medicamentos

Anaphylaxis: A Decade of a Nationwide Allergy Society Registry.

BACKGROUND AND OBJECTIVES: Anaphylaxis is an acute, life-threatening, multiorgan hypersensitivity reaction. Objective: The aim of this study was to identify the causes of anaphylaxis in Portugal in order to improve our knowledge of epidemiology and management. METHODS: We implemented a nationwide notification system for anaphylaxis over a 10-year period, with voluntary reporting by allergists. Data on 1783 patients with anaphylaxis were included. Etiopathogenesis, manifestations, and clinical management were characterized in detail for both children and adults. RESULTS: The mean age was 32.7 (20.3) years, and 30% were under 18 years of age; 58% were female. The mean age at the first anaphylaxis episode was 27.5 (20.4) years (ranging from 1 month to 88 years). The main culprits of anaphylaxis were foods (48%), drugs (37%) (main trigger in adults, 48%), and hymenoptera venom (7%). The main culprit foods were shellfish (27%), fresh fruit (17%), cow's milk (16%), tree nuts (15%), fish (8%), egg (7%), and peanut (7%). The main drugs were nonsteroidal anti-inflammatory drugs (43%), antibiotics (39%), and anesthetic agents (6%). Other causes included exercise (3%), latex (2%), cold-induced anaphylaxis (2%), and idiopathic anaphylaxis (2%). Most patients (80%) were admitted to the emergency department; only 43% received adrenaline. Anaphylaxis recurred in 41% of patients (21% with ≥3 anaphylactic episodes); 7% used an adrenaline autoinjector device. CONCLUSION: Food is the leading cause of anaphylaxis in Portugal, while drugs were the main elicitors in adults. We emphasize undertreatment with adrenaline and recurrent episodes, highlighting the need to improve diagnostic and therapeutic approaches to anaphylaxis.

Evolution of Api m10 Specific IgE and IgG4 After One Year of Bee Venom Immunotherapy.

Summary: Background. Bee-venom (BV) anaphylaxis can be life-threatening, requiring treatment with BV immunotherapy (bVIT). Different molecular profiles may be associated with different outcomes after bVIT. Methods. In 19 patients with BV anaphylaxis, sensitized both to Api m1 and Api m10, we evaluated sIgE and sIgG4 Api m1 and Api m10 levels before and after 1 year bVIT.Results.7 patients (37%) had higher baseline Api m10 than Api m1 sIgE levels (Api m10 predominant). bVIT reduced sIgE to both components but sIgG4 levels were increased only for Api m1. 5 patients (2 in the Api m10 predominant group) were re-stung without anaphylaxis. Conclusions. Although there was no increase in Api m10 sIgG4 levels after 1 year bVIT, we did not observe relevant differences in other outcomes between patients with predominant Api m1 or Api m10 sensitization.

Contribution of in vivo and in vitro testing for the diagnosis of local allergic rhinitis

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Salmon roe as an emerging allergen in western countries

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Severe bronchiectasis in a patient with common variable immunodeficiency.

BACKGROUND: Bronchiectasis are common in Common Variable Immunodeficiency. These patients are prone to infection, leading to progressive lung destruction and accelerated FEV1 decline. CLINICAL CASE: 40 year-old man, with recurrent respiratory infections, autoimmunity and diarrhea since age 7. At 17 CVID was diagnosed and IVIgG was started. During the following years, respiratory symptoms progressively worsened and bronchiectasis was found on thoracic computed tomography. Bronchoscopy revealed Pseudomonas aeruginosa in bronchoalveolar lavage and bronchial secretions cultures. Eradication therapy led to clinical improvement. DISCUSSION: This case report stresses the importance of regular microbiological screening and appropriate antibiotherapy. Early/aggressive treatment may significantly impact on patients' evolution.

Longitudinal study of the expression of FcεRI and IgE on basophils and dendritic cells in association with basophil function in two patients with severe allergic asthma treated with Omalizumab.

Severe asthma is a challenging disease, and omalizumab has been an important tool to help clinicians address more efficiently this problem. Besides reduction of free and total serum IgE levels, there are a number of other immunologic effects of omalizumab that may be of relevance in its therapeutic action. We report two mite-allergic severe asthmatic patients successfully treated with omalizumab for one year. Clinically, patients improved gradually, with no further need for systemic steroids or emergency department visits during that treatment period, and with Asthma Control Test (ACT) scores showing controlled disease, although pulmonary function didn't show any significant improvement. Immunologically, we observed marked down-regulation of surface IgE and FcεRI on basophils, plasmacytoid and myeloid dendritic cells, as well as a reduction of basophil activation after specific allergen stimulation. These effects were clearly evident immediately after one month but were enhanced at 3, 6 and 12 months of omalizumab treatment, suggesting an advantage to continuing this therapy, and raising the hypothesis of some markers being useful to assess immunological responses to omalizumab, which could assist in the clinician's decision to stop or to restart this treatment.

Prevalence and classification of rhinitis in preschool children in Portugal: a nationwide study.

BACKGROUND: Information on rhinitis epidemiology in preschool children is scarce. OBJECTIVES: To estimate, in children with 3-5 years of age, current rhinitis prevalence, to describe rhinitis symptoms and associated sociodemographic characteristics, and to report allergic rhinitis and its impact on asthma (ARIA) classification. METHODS: Cross-sectional, nationwide, population-based study including children aged 3-5 years. A multistep selection strategy was used to obtain a representative sample. Data were collected at kindergartens and parish centers by face-to-face interview to caregivers, using an adapted ISAAC questionnaire. 'Current rhinitis' (CR) was defined as the presence of ≥1 rhinitis symptom (repeated sneezing and/or itchy nose, blocked nose for >1 h, or runny nose without having a cold/flu) in the last year. Rhinitis lasting ≥4 days/week and ≥4 consecutive weeks was classified as persistent. Moderate-severe disease interfered 'a moderate amount' or 'a lot' in daily activities. RESULTS: Five thousand and eighteen children were included. CR prevalence was 43.4% [95% CI (42.0-44.8%); n = 2179] and that of physician-diagnosed rhinitis was 11.7% [95% CI (10.8-12.6%); n = 588]. Of children with CR, 67% reported blocked nose, 48% rhinorrhea, and 46% sneezing/itchy nose. Considering ARIA classification, 30% of the population had mild intermittent, 3% mild persistent, 7% moderate-severe intermittent, and 3% moderate-severe persistent rhinitis. Children with CR had more current wheezing, physician-diagnosed asthma, self-reported food allergy, and family history of allergic disease. These characteristics were also associated with persistent and moderate-severe rhinitis. CONCLUSIONS: This is the first nationwide population-based study reporting rhinitis prevalence and ARIA classification in preschool children. In this age-group, rhinitis is frequent and underdiagnosed. About 25% children with rhinitis presented moderate-severe disease.

Correlation and discriminant analysis between clinical, endoscopic, thoracic X-ray and bronchoalveolar lavage fluid cytology scores, for staging horses with recurrent airway obstruction (RAO).

As recurrent airway obstruction (RAO) is progressive and as medical history is frequently unknown by owners, it's important to suggest a score model to characterize RAO stages for a more accurate diagnosis and treatment. The authors correlated clinical (CS), endoscopic (ES), thoracic X-ray (XRS) and bronchoalveolar lavage fluid (BALFS) scores in horses with RAO, in an attempt to establish relevance of each factor's contribution for the characterization of RAO stages and to suggest a staging method. Thirty horses with RAO and ten healthy controls were studied. Pearson correlation coefficients were determined between CS, ES, XRS and BALFS. Only significant correlation coefficients (>0.60) were considered. One way variance analyses were used to compare the two groups. A discriminant analysis model was adjusted on the RAO staging method suggested. There was a significant correlation coefficient between the CS cough, nostril flare and abdominal lift, all the mucus ES (0.61-0.84), the XRS interstitial pattern, bronchial radiopacity and thickening and tracheal thickening (0.67-0.78) and the BALFS neutrophil percentages (0.63-0.84). These variables (e.g., cough) which presented a significant correlation coefficient were considered relevant and chosen for a score model to characterize RAO stages. The ten healthy controls were attributed stage 0 and the 30 RAO horses were attributed stages 1 (4 horses), 2 (7 horses), 3 (10 horses) and 4 (9 horses). There was also a significant correlation coefficient between all the relevant variables and the RAO stage (0.61-0.89). Furthermore, discriminant analysis of the RAO staging method showed 92.5% of original grouped cases and 85.0% of cross-validated grouped cases correctly classified, having confirmed major contribution of the same variables that had significant correlation coefficients. Even though further confirmation by lung functional testing is desirable, the significant correlation between relevant variables and RAO stage and the results of RAO staging discriminant analysis support the suggestion of our score model for the characterization of RAO stages.

Validation of a questionnaire (CARAT10) to assess rhinitis and asthma in patients with asthma.

BACKGROUND AND AIM: The Control of Allergic Rhinitis and Asthma Test (CARAT) was developed to be used in the concurrent management of these diseases, as recommended by the Allergic Rhinitis and its Impact on Asthma (ARIA) guidelines. However, it was necessary to statistically identify and remove redundant questions and to evaluate the new version's factor structure, internal consistency and concurrent validity. METHODS: In this cross-sectional study 193 adults with allergic rhinitis and asthma from 15 outpatient clinics in Portugal were included. The CARAT questionnaire was reduced using descriptive analysis, exploratory factor analysis and internal consistency. Spearman's correlations were used to compare the CARAT scores with a medical evaluation and other measures of control, including the Asthma Control Questionnaire and symptoms' visual analogue scales. The performance against physician rating of control was summarized using the area under the curve (AUC) from receiver operating characteristic analysis. In addition, CARAT was compared with the physician's decision to reduce, maintain or increase treatment. RESULTS: The reduced version has 10 questions and 2 factors (CARAT10). The Cronbach's alpha was 0.85. All correlation coefficients of CARAT10 and factors with the different measures of control met the a priori predictions, ranging from 0.58 to 0.79. The AUC was 0.82. For the physician's decision groups of reduce, maintain or increase treatment, the mean (IC95%) scores of CARAT10 were 24 (21.4;26.6), 21 (19.4;21.9) and 15 (13.6;16.5), respectively. CONCLUSION: CARAT10 has high internal consistency and good concurrent validity, making it useful to compare groups in clinical studies.

Anaphylaxis to pine nut: cross-reactivity to Artemisia vulgaris?

The use of pine nuts, the seeds of Pinus pinea, is on the increasing in the modern Mediterranean diet. Little more than 20 cases of allergy to this tree nut have been published, and cross-reactivity with pine pollen, peanut and almond has already been reported. We describe the case of a young boy with several episodes of anaphylaxis after pine nut ingestion. Specific IgE to pine nut and Artemisia vulgaris was demonstrated by skin prick tests and in vitro determination of specific IgE, although no IgE to pine pollen or other nuts was detected. Immunoblotting of Artemisia vulgaris and pine nut revealed two matching diffuse bands, just below 14 kDa and 30 kDa. The ImmunoCAP inhibition assays showed complete inhibition of pine nut specific IgE after serum incubation with Artemisia vulgaris extract. As far as we know, this is the first reported case of documented cross-reactivity between pine nut and Artemisia vulgaris.

Anaphylaxis to pine nut: cross-reactivity to Artemisia vulgaris?

The use of pine nuts, the seeds of Pinus pinea, is on the increasing in the modern Mediterranean diet. Little more than 20 cases of allergy to this tree nut have been published, and cross-reactivity with pine pollen, peanut and almond has already been reported. We describe the case of a young boy with several episodes of anaphylaxis after pine nut ingestion. Specific IgE to pine nut and Artemisia vulgaris was demonstrated by skin prick tests and in vitro determination of specific IgE, although no IgE to pine pollen or other nuts was detected. Immunoblotting of Artemisia vulgaris and pine nut revealed two matching diffuse bands, just below 14 kDa and 30 kDa. The ImmunoCAP® inhibition assays showed complete inhibition of pine nut specific IgE after serum incubation with Artemisia vulgaris extract. As far as we know, this is the first reported case of documented cross-reactivity between pine nut and Artemisia vulgaris

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Immediate and delayed hypersensitivity to latex in a social caregiver

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