The diagnostic workup for systemic mastocytosis differs from consensus recommendations: Results of a worldwide survey.

Objective: Mastocytosis is a complex disorder affecting various organs. The diagnostic workup can be challenging and requires a multidisciplinary approach including the use of uncommon tests. To assess mastocytosis management around the globe, we conducted the first worldwide online survey for physicians. Methods: A 21-item questionnaire was sent out to the members of the World Allergy Organization (WAO), the Global Allergy and Asthma European Network (GA2LEN), the Urticaria (UCARE) and Angioedema (ACARE) Centers of Reference and Excellence, the German Society of Allergology and Clinical Immunology (DGAKI), and the European Mast Cell and Basophil Research Network (EMBRN) in April-June 2021. Results: Across 628 respondents from 79 countries 87.7% and 9.7% of physicians were allergists/clinical immunologists and/or dermatologists. Participating physicians were from all regions of the world (Europe, EU: 41.6%; North America, NA: 24.8%; Latin America, LA: 14.5%; Asia-Pacific, AP: 12.6%; and Africa/Middle East, AME: 6.5%). Only 2.2% of respondents worked at Specialized Mastocytosis Centers (SMCs) in North America or European Union. Physicians reported caring for 4 patients with mastocytosis per year, with higher numbers in European Union and Asia Pacific (5/year) compared to Latin America (2/year). Dermatologists and physicians who work at SMCs reported higher patient numbers (15/year and 80/year, respectively). Suspicion of mastocytosis in the allergology and dermatology community is commonly driven by anaphylaxis (82.9%), mastocytosis skin lesions (82.1%), or elevated tryptase levels (76.6%). Osteoporosis and gastrointestinal symptoms less often prompted suspicion of mastocytosis (21.4% and 49.9%, respectively). World Health Organisation (WHO)-diagnostic criteria and classification, regardless of the region, are only used by about 50% of physicians, with higher rates for SMCs (83.3%). Serum tryptase, bone marrow biopsy, and KIT D816V mutation analysis are included in the diagnostic workup by 90.9%, 61.5%, and 58.4% of physicians, respectively. The biggest challenges for the management of mastocytosis are the lack of more effective treatment options (51.1%), missing multidisciplinary networks (47.1%), and the lack of experience of specialists from other disciplines (39.0%). Conclusions: The diagnostic workup for mastocytosis differs from consensus recommendations and varies between regions. This may be improved by establishing active multidisciplinary networks, increasing access to diagnostic procedures, consistently applying WHO criteria, and developing new Mastocytosis Centers of Reference and Excellence.

Oral mite anaphylaxis (pancake syndrome) in Peru: an underestimated problem? / Anafilaxia por ingesta de ácaros (Síndrome da panqueca) no Peru: um problema subestimado?

Sabe-se que a urticária e o angioedema apresentam diferentes etiologias, pois podem ser de natureza alérgica, infecciosa, autoimune ou espontânea. Em episódios únicos ou recorrentes, deve-se considerar um alérgeno desencadeante oculto, como os ácaros de poeira doméstica (APDs). Vários relatos demonstraram que farinhas contaminadas com APDs podem causar urticária e angioedema, incluindo reações alérgicas graves com risco de vida quando ingeridos em grandes quantidades provenientes de farinha de trigo armazenada. Neste estudo, relatamos os achados clínicos de 31 pacientes, incluindo casos de anafilaxia após ingestão de farinha contaminada com ácaros. Também encontramos uma relação entre uma história clínica de hipersensibilidade a anti-inflamatórios não esteroides e síndrome de anafilaxia por ingesta de ácaros em pacientes atópicos, consistente com a teoria de uma "nova tríade do ácido acetilsalicílico", conforme publicado anteriormente, e agora sendo descrito pela primeira vez no Peru.

Urticaria and angioedema are known to have different etiologies, as they can be allergic, infectious, autoimmune, or spontaneous in nature. In single or recurrent episodes, a hidden triggering allergen should be considered, such as house dust mites (HDMs). Several reports have demonstrated that flours contaminated with HDMs can cause urticaria and angioedema, including severe lifethreatening allergic reactions when ingested in large quantities from stored wheat flour. In this study, we report the clinical findings in 31 patients, including cases of anaphylaxis after the ingestion of mite-contaminated flour. We also found a relationship between a clinical history of hypersensitivity to nonsteroidal anti-inflammatory drugs and oral mite anaphylaxis syndrome in atopic patients, consistent with the theory of a "new aspirin triad," as previously published, and now being described for the first time in Peru.

Diretriz Latino-americana sobre o Diagnóstico e Tratamento da Alergia Ocular ­ Em nome da Sociedade Latinoamericana de Alergia, Asma e Imunologia (SLAAI) / Latin American Guideline on the Diagnosis and Treatment of Ocular Allergy ­ On behalf of the Latin American Society of Allergy, Asthma and Immunology (SLAAI)

A alergia ocular, também conhecida como conjuntivite alérgica (CA), é uma reação de hipersensibilidade mediada por imunoglobulina E (IgE) do olho desencadeada por aeroalérgenos, principalmente ácaros da poeira doméstica e pólen de gramíneas. Os sintomas geralmente consistem em prurido ocular ou periocular, lacrimejamento e olhos vermelhos que podem estar presentes durante todo o ano ou sazonalmente. A alergia ocular tem frequência elevada, é subdiagnosticada e pode ser debilitante para o paciente. É potencialmente danosa para a visão, nos casos em que ocasiona cicatrização corneana grave, e na maioria dos pacientes associa-se a outros quadros alérgicos, principalmente rinite, asma e dermatite atópica. É classificada em conjuntivite alérgica perene, conjuntivite alérgica sazonal, ceratoconjuntivite atópica e ceratoconjuntivite vernal. O diagnóstico procura evidenciar o agente etiológico e a confirmação se dá pela realização do teste de provocação conjuntival. O tratamento baseia-se em evitar o contato com os desencadeantes, lubrificação, anti-histamínicos tópicos, estabilizadores de mastócitos, imunossupressores e imunoterapia específica com o objetivo de obter o controle e prevenir as complicações da doença.

Ocular allergy, also known as allergic conjunctivitis, is an immunoglobulin E-mediated hypersensitivity reaction of the eye triggered by airborne allergens, primarily house dust mites and grass pollen. Symptoms usually consist of ocular or periocular itching, watery eyes, and red eyes that may be present year-round or seasonally. Ocular allergy has a high frequency, is underdiagnosed, and can be debilitating for the patient. It is potentially harmful to vision in cases of severe corneal scarring, and in most patients, it is associated with other allergic conditions, especially rhinitis, asthma, and atopic dermatitis. It is classified as perennial allergic conjunctivitis, seasonal allergic conjunctivitis, atopic keratoconjunctivitis, and vernal keratoconjunctivitis. Diagnosis seeks to identify the etiologic agent, and confirmation is given by conjunctival provocation testing. Treatment is based on avoiding contact with triggers, lubrication, topical antihistamines, mast cell stabilizers, immunosuppressants, and specific immunotherapy with the aim of achieving control and preventing disease complications.

The Use of Contrast Agents in Interventional Pain Procedures: A Multispecialty and Multisociety Practice Advisory on Nephrogenic Systemic Fibrosis, Gadolinium Deposition in the Brain, Encephalopathy After Unintentional Intrathecal Gadolinium Injection, and Hypersensitivity Reactions.

This Practice Advisory presents a comprehensive and evidence-based set of position statements and recommendations for the use of contrast media in interventional pain procedures. The advisory was established by an international panel of experts under the auspices of 11 multinational and multispecialty organizations based on a comprehensive review of the literature up to December 31, 2019. The advisory discusses the risks of using gadolinium-based contrast agents. These include nephrogenic systemic fibrosis, gadolinium brain deposition/retention, and encephalopathy and death after an unintentional intrathecal gadolinium injection. The advisory provides recommendations on the selection of a specific gadolinium-based contrast agent in patients with renal insufficiency, those who had multiple gadolinium-enhanced magnetic resonance imaging examinations, and in cases of paraspinal injections. Additionally, recommendations are made for patients who have a history of mild, moderate, or severe hypersensitivity reactions to contrast medium.

Latin American chronic urticaria registry (CUR) contribution to the understanding and knowledge of the disease in the region.

Chronic urticaria (CU) has a widespread spectrum on causal or exacerbating factors, clinical manifestations, therapeutic response and quality of life affectation. Registries are useful tools in several real-life diagnosis and management approach.We aimed to evaluate the characteristics of CU patients living in Latin America through an original cross-sectional registry with data entered by regional allergologists. RESULTS: Three hundred patients were included, being 72% female, with median age of 36 years (1-85) and 20 months of CU median evolution time. The cause of CU was reported as unknown in 72% of them.Thirty-nine percent of suspected cases presented positive serology for Mycoplasma, positive autologous serum skin test (ASST) was reported in 47%, and occasional presence of thyroid or antinuclear autoantibodies and parasites. The impact of pruritus in their quality of life was moderate to severe in 60% of patients, with almost 3 out of four patients having partial or lack of urticaria control with anti-histamines. CONCLUSIONS: Our registry provides retrospective data on the real-life assistance of a large number of patients from the region. Continuous search for associated conditions and better treatment possibilities are needed, in order to control the significant impact on quality of life and the length of disease.

Omalizumab vs. placebo in the management of chronic idiopathic urticaria: a systematic review.

OBJECTIVES: To examine the evidence derived from randomized controlled clinical trials on the efficacy and safety of omalizumab compared to placebo in controlling symptoms of chronic idiopathic urticaria/chronic spontaneous urticaria (CIU/CSU). DATA SOURCE: The electronic databases PubMed, Medline, EMBASE, Biomed Central, The Cochrane Central Register of Controlled Trials (CENTRAL), Wiley, OVID, and HighwirePress were reviewed. The date limit was set to May 31th, but it was extended to September 30th of 2014 due to a new publication. No language restriction was used. The articles included were randomized trials controlled with placebo in individuals older than 12 years diagnosed with CIU/CSU refractory to conventional treatment, the intervention being, omalizumab at different doses, and the comparison, placebo. The primary outcome was symptom improvement according to the weekly score of urticaria severity (UAS7), the itch severity score (ISS), the weekly score of number of urticarial lesions, the dermatology life quality index, and the chronic urticaria quality of life questionnaire (CU-QoL). Databases were searched using the following Mesh or EMTREE key words including as intervention "omalizumab" or "humanized monoclonal antibody," compared to placebo and the disease of interest "urticaria" or "angioedema". The title, abstract and article were reviewed by two independent investigators, according to the selection criteria in each of the databases. An assessment of the quality of the articles was performed according to the bias tool from the studies of the Cochrane Collaboration. Information such as author data, date of study, number of participants, interventions, dose and frequency of administration, comparison, time of follow-up, measurements of weekly score of urticaria activity, pruritus severity score, weekly urticarial lesions, percentage of angioedema and post-treatment change were extracted. Frequency of adverse events and the ones suspected to be caused by the intervention drug were included. RESULTS: 770 records were identified in all databases described. 720 were eliminated for failing to meet the inclusion criteria in the first review or for duplicate records. 24 articles were reviewed by abstract, 18 additional articles were further removed, leaving 6 records for inclusion. An experimental study was excluded because it wasn't randomized. Five studies were finally included, with 1117 patients, of these 831 received a dose of omalizumab of 75 mg (183 patients, 16.38%), 150 mg (163 patients, 14.59%), 300 mg (437 patients, 39.12%) or 600 mg (21 patients, 1.8%), as a single dose, or every 4 weeks until 24 weeks maximum. The average age was 42.07 years, predominantly female gender and white ethnicity. It was observed that the use of omalizumab 300 mg lowered the weekly scores of urticarial activity in 19.9 vs. 6.9 on placebo (p <0.01), 19 vs 8.5 and 20.7 vs 8.01 in three studies, the weekly ISS (-9.2 vs. - 3.5, p <0.001, -9.8 vs -5.1 p < 0.01, -8.6 vs -4.0 and -9.4 vs -3.63 p <0.001 in four studies), and the percentage of angioedema-free days (omalizumab 95.5% vs. placebo 89.2% p <0.001, and 91.95% vs. 88.1% p <0.001 in two of the studies respectively). LIMITATIONS: The different doses used throughout the study, time of administration and follow-up periods ranged from single dose to monthly dose for 24 weeks. Therefore no meta-analysis of the review was conducted. CONCLUSIONS AND IMPLICATIONS OF THE MAIN FINDINGS: Despite the limitations, it is considered that omalizumab 300 mg is effective in treating chronic idiopathic urticaria refractory to H1 antihistamines. Further studies are required to determine the duration of effective treatment. REGISTRATION NUMBER OF THE SYSTEMATIC REVIEW: CRD42014010029 (PROSPERO. International Prospective Register of Systematic Reviews).

Allergy training and immunotherapy in Latin America: results of a regional overview.

BACKGROUND: One main practice gap in allergology that has been detected in several regions of the world is the application of specific immunotherapy (SIT). The prescription and practice of SIT should characterize allergologic specialists, but there are regional discrepancies in such practice. A detailed knowledge of the regulatory and legislation aspects and drawbacks would help improve and harmonize SIT practice. OBJECTIVE: To describe in Latin America the level of allergy training and the characteristics of the use of SIT, including the medical and legal aspects. METHODS: Three sources were used: a 24-item questionnaire sent to 22 allergologic leaders in 11 Latin American countries, 2 face-to-face meetings, and information from health authorities involved in the approval of medical substances. RESULTS: In 56% of countries, the specialty of allergology is a third-level care specialty and/or a subspecialty. Two countries have a special training program for pediatric allergists. Passing a board examination is mandatory in 3 countries, and recertification every 2 to 5 years occurs without examination. Sublingual and subcutaneous SITs are available in all Latin American countries. No legislation restricts SIT prescription and it can be performed by nonspecialists in 7 of 11 countries. In 90% of countries, allergists use allergen extracts from the United States (subcutaneous immunotherapy) and Europe (sublingual and subcutaneous immunotherapies), and 50% also manufacture extracts locally. Only 1 country has legal requirements for the quality of raw materials. CONCLUSION: The present analysis helps to identify gaps in the field of allergologic training and SIT in Latin America, many of them amendable.

Anaphylaxis in Latin America: a report of the online Latin American survey on anaphylaxis (OLASA).

OBJECTIVES: The aims of the Online Latin American Survey of Anaphylaxis (OLASA) were to identify the main clinical manifestations, triggers, and treatments of severe allergic reactions in patients who were seen by allergists from July 2008 to June 2010 in 15 Latin American countries and Portugal (n =634). RESULTS: Of all patients, 68.5% were older than 18 years, 41.6% were male, and 65.4% experienced the allergic reaction at home. The etiologic agent was identified in 87.4% of cases and predominantly consisted of drugs (31.2%), foods (23.3%), and insect stings (14.9%). The main symptom categories observed during the acute episodes were cutaneous (94.0%) and respiratory (79.0%). The majority of patients (71.6%) were treated initially by a physician (office/emergency room) within the first hour after the reaction occurred (60.2%), and 43.5% recovered in the first hour after treatment. Most patients were treated in an emergency setting, but only 37.3% received parenteral epinephrine alone or associated with other medication. However, 80.5% and 70.2% were treated with corticosteroids or antihistamines (alone or in association), respectively. A total of 12.9% of the patients underwent reanimation maneuvers, and 15.2% were hospitalized. Only 5.8% of the patients returned to the emergency room after discharge, with 21.7% returning in the first 6 hours after initial treatment. CONCLUSION: The main clinical manifestations of severe allergic reactions were cutaneous. The etiologic agents that were identified as causing these acute episodes differed according to age group. Following in order: drugs (31.2%), foods (23.3% and insect stings (14.9%) in adults with foods predominance in children. Treatment provided for acute anaphylactic reactions was not appropriate. It is necessary to improve educational programs in order to enhance the knowledge on this potentially fatal emergency.

Anaphylaxis in Latin America: a report of the online Latin American survey on anaphylaxis (OLASA)

OBJECTIVES: The aims of the Online Latin American Survey of Anaphylaxis (OLASA) were to identify the main clinical manifestations, triggers, and treatments of severe allergic reactions in patients who were seen by allergists from July 2008 to June 2010 in 15 Latin American countries and Portugal (n =634). RESULTS: Of all patients, 68.5 percent were older than 18 years, 41.6 percent were male, and 65.4 percent experienced the allergic reaction at home. The etiologic agent was identified in 87.4 percent of cases and predominantly consisted of drugs (31.2 percent), foods (23.3 percent), and insect stings (14.9 percent). The main symptom categories observed during the acute episodes were cutaneous (94.0 percent) and respiratory (79.0 percent). The majority of patients (71.6 percent) were treated initially by a physician (office/emergency room) within the first hour after the reaction occurred (60.2 percent), and 43.5 percent recovered in the first hour after treatment. Most patients were treated in an emergency setting, but only 37.3 percent received parenteral epinephrine alone or associated with other medication. However, 80.5 percent and 70.2 percent were treated with corticosteroids or antihistamines (alone or in association), respectively. A total of 12.9 percent of the patients underwent reanimation maneuvers, and 15.2 percent were hospitalized. Only 5.8 percent of the patients returned to the emergency room after discharge, with 21.7 percent returning in the first 6 hours after initial treatment. CONCLUSION: The main clinical manifestations of severe allergic reactions were cutaneous. The etiologic agents that were identified as causing these acute episodes differed according to age group. Following in order: drugs (31.2 percent), foods (23.3 percent and insect stings (14.9 percent) in adults with foods predominance in children. Treatment provided for acute anaphylactic reactions was not appropriate. It is necessary to improve educational programs in order to enhance the knowledge on this potentially fatal emergency.

[Allergic rhinitis update and its impact on asthma (ARIA 2008). Latin American perspective]. / Actualización de rinitis alérgica y su impacto en el asma (ARIA 2008). La perspectiva latinoamericana.

Rhinitis is the most frequent respiratory disease in most countries of the world. It is estimated that 600 million people suffer this condition. Allergic rhinitis is a public health problem at global level. Patients who suffer allergic rhinitis have from mild to annoying nasal symptoms which affect quality of life, cause sleep disorders, scholar and workplace absenteeism, and health expenditure. Rhinitis is frequently associated to co-morbidities such as sinusitis, otitis media, and especially asthma. Rhinitis is under-diagnosed and under-treated worldwide and also in Latin American countries. ARIA is the very first evidence-based guideline for the diagnosis and treatment of rhinitis with focus in its co-morbidities (2001), especially asthma published in 2001. In 2008 an update was published. ARIA recommends an integrative approach for management; including anti-histamines (second generation), intra-nasal corticosteroids, anti-leukotrienes and immunotherapy. It also provides a questionnaire to evaluate asthma and its severity in those patients suffering rhinitis. The prevalence of allergic rhinitis is quite high in Latin American countries and in recent years a great insight on the burden of this condition has been gained.