Expert Review and Consensus on the Treat-to-Target Management of Hereditary Angioedema: From Scientific Evidence to Clinical Practice.

BACKGROUND: Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is a rare disease characterized by swelling episodes. It affects quality of life (QOL) and can be fatal when the upper airways are involved. Treatment is individualized, with therapeutic options including on-demand treatment (ODT) and short- and long-term prophylaxis (STP, LTP). However, available guidelines are not always clear about the selection of treatment, the goals of treatment, or how achievement of these goals is assessed. OBJECTIVE: To review available evidence for the management of HAE-C1INH and build a Spanish expert consensus to steer management towards a treat-to-target approach, while addressing some of the less clear aspects of the Spanish guidelines. METHODS: We reviewed the literature on the treat-to-target management of HAE-C1INH, focusing on treatment selection and goals and the tools available to assess whether the goals have been achieved. We discussed the literature based on clinical experience and drew up 45 statements on undefined management aspects. A panel of 53 HAE experts validated the statements through a 2-round Delphi process. RESULTS: The goals for ODT and STP are to minimize the morbidity and mortality of attacks and to prevent attacks caused by known triggers, respectively, while the main goal of LTP is to decrease the rate, severity, and duration of attacks. Furthermore, when prescribing, clinicians should consider the reduction in adverse effects, while increasing patient QOL and satisfaction. Appropriate instruments for assessing achievement of treatment goals are also indicated. CONCLUSIONS: We provide recommendations on previously unclear aspects of HAE-C1INH management with ODT, STP, and LTP, focusing on clinical and patient-oriented goals.

Treating COVID-19: Review of Drug Hypersensitivity Reactions.

The disease caused by the new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), ie, coronavirus disease 2019 (COVID-19), has become a global pandemic since it was first reported in Wuhan, China in December 2019. Its severe clinical manifestations, which often necessitate admission to intensive care units, and high mortality rate represent a therapeutic challenge for the medical community. To date, no drugs have been approved for its treatment, and various therapeutic options are being assayed to address the pathophysiological processes underlying the clinical manifestations experienced by patients. New and old drugs administered as monotherapy or in combination to immunologically compromised patients may favor the development of adverse drug reactions, including drug hypersensitivity reactions, which must be identified and managed accordingly. Given the lack of herd immunity and the high rate of viral contagion, new cases are expected to emerge in the coming months. Thus, the probability of more adverse reactions or even new clinical manifestations may increase in parallel. Allergists must receive updated information on these treatments, as well as on the management of possible drug hypersensitivity reactions.

Primula dermatitis.

We report the cases of 2 patients diagnosed as having primula dermatitis. Both patients were housewives who had been exposed to primula plants. Although only one related her clinical manifestations with looking after plants, the symptoms of both remitted when contact with primula was avoided. The diagnosis was carried out with a patch test concentration of 0.01% primin pet. As primula dermatitis has variable clinical manifestations that can not easily be related to contact allergy, we emphasize the need to include synthetic primin in our standard patch test series.

[Bird-egg syndrome in children]. / Síndrome ave-huevo en niños.

Allergy to egg is highly frequent in childhood. In general, children allergic to egg react principally to the ingestion of egg white. Egg yolk contains various proteins but the major allergens are contained in egg white. The principal allergens are ovalbumin, ovomucoid, ovotransferrin, and lysozyme. These proteins have been sequenced. In some cases, a relationship between type I hypersensitivity with respiratory symptoms due to bird antigens and allergy to egg yolk has been described. This association is known as bird-egg syndrome, which is caused by sensitization to chicken serum albumin (alpha -livetin) and is characterized by the development of respiratory and gastrointestinal symptoms after egg intake or after contact with bird antigens. The initial symptoms are usually asthma with or without rhinoconjunctivitis due to contact with birds. Individuals first become sensitized to bird proteins (feathers, excrement, serum and meat) and subsequently develop egg allergy. Although bird-egg syndrome has been described principally in adults, especially in women, it can also affect children in whom the syndrome presents certain differentiating characteristics in relation to the more common sensitization to egg white. Gastrointestinal and respiratory symptoms are more common than cutaneous symptoms and sensitization to egg yolk is more frequent than that to egg white. In children with allergy to birds and egg, egg allergy is usually more persistent; tolerance is not always achieved and develops later. Sensitization to other aeroallergens is also greater in individuals with allergy to birds and egg. Sensitization to egg sometimes precedes respiratory sensitization to bird proteins, a process known as bird-egg syndrome. By way of example, the case of a child who clinically presented bird-egg syndrome is presented at the end of this review.