Symptoms Of Angioedema Research Articles

Chronic Spontaneous Urticaria-New Predictor on the Horizon?

Chronic urticaria is one of the most common diseases in allergology and dermatology practice with unclear causes of occurrence. Background: Some studies emphasize the correlation between inflammation in chronic urticaria and disturbed intestinal microbiota. It raises the question about the role of some intestine-related substances in the pathogenesis of urticaria as well as their potential role as disease predictors. Calprotectin is an acute-phase protein with a well-established diagnostic position in the field of gastroenterology. There are some data on the relationship between this parameter and gut microbiota. The major aim of this preliminary study is to investigate whether calprotectin can be potentially taken into account as a disease course predictor in urticaria. Methods: We included in our study 54 chronic spontaneous urticaria (CSU) patients (of whom 26 manifested the symptoms of recurrent angioedema) and 29 patients allergic to Hymenoptera venom for the reference group (in these patients, before venom immunotherapy induction, full diagnostics is performed including intestinal problems). Disease activity in the CSU patients was assessed using the Urticaria Activity Score (UAS) and the disease control in this group was assessed with the Urticaria Control Test (UCT). Moreover, we analyzed fecal and serum calprotectin concentrations. Results: Positive correlation was found only between the values of serum calprotectin concentration and the control level of CSU symptoms with the lack of other relations. Conclusions: Our results do not supply unequivocal evidence for calprotectin as a potential marker of CSU course, though this concept, in the light of growing evidence for microbiota's role in urticaria, requires further research.

Analysis of disease burden in patients with hereditary angioedema from Japan by patient-reported outcomes.

Hereditary angioedema (HAE) symptoms can vary greatly. Disease burden evaluation is essential for providing adequate treatments for patients. Patient-reported outcome measures (PROMs), including the 12-Item Short Form Health Survey (SF-12), the Angioedema Quality of Life (AE-QoL), the Hospital Anxiety and Depression Scale (HADS), and the Work Productivity and Activity Impairment: Specific Health Problem (WPAI:SHP) questionnaires, were collected in 2021, before modern medications for long-term prophylaxis (LTP) of HAE were licensed in Japan. Patients also reported their HAE attack frequency as "annual" (several attacks annually), "monthly" (several attacks monthly) or "weekly" (several attacks weekly). Multiple linear regression analyses were conducted on the relationship between independent parameters (sex, age, attack frequency, HAE type, and HADS scores) and dependent parameters (AE-QoL and SF-12 scores). Fifty-four patients reported PROMs. All PROMs showed substantial health-related quality of life (HRQoL) impairment. Overall, the higher the attack frequencies, the greater the reported impairment in the PROMs tended to be. In multiple linear regression analyses, higher AE-QoL Fatigue/Mood and Fears/Shame domain scores (greater impairment) were associated with higher HADS anxiety subscale scores; higher AE-QoL total scores (greater HRQoL impairment) and lower SF-12 Physical and Mental Health Composite scores (greater general health impairment) were associated with higher HADS depression subscale scores. Patients with monthly or weekly HAE attacks reported numerically low absenteeism and numerically high presenteeism and work productivity loss as measured by the WPAI:SHP questionnaire. In this study, conducted before modern LTP options were available in Japan, patients with HAE reported notable impairment in HRQoL and work productivity. Weekly or monthly HAE attack frequencies were associated with a high disease burden. Furthermore, a substantial number of patients reported notable fatigue/mood impairment as measured by the AE-QoL and depression as measured by the HADS regardless of attack frequency. These results provide a basis for future studies evaluating the effect of LTP on the clinical manifestations and HRQoL in patients with HAE.

Lanadelumab in Patients 2 to Less Than 12 Years Old With Hereditary Angioedema: Results From the Phase 3 SPRING Study

Symptoms of hereditary angioedema (HAE) often first occur during childhood, and HAE attacks in children can be severe and substantially affect health-related quality of life (HRQoL). However, there are no approved long-term prophylaxis treatments for children aged less than 6 years. The SPRING Study (NCT04070326) evaluated the safety, pharmacokinetics, and efficacy of lanadelumab and HRQoL in patients aged 2 to less than 12 years. Over 52 weeks of treatment, patients aged 2 to less than 6 years received lanadelumab 150 mg every 4 weeks (Q4W) and patients aged 6 to less than 12 years received 150 mg every 2 weeks (Q2W) but could switch to Q4W if they were attack-free for 26 weeks. We enrolled 21 patients (aged 2 to less than 6 years: n= 4; aged 6 to less than 12 years: n= 17), 20 of whom completed the study. There were no reported serious treatment-emergent adverse events or discontinuations resulting from such events. Treatment-emergent adverse events were reported for 17 patients (81.0%). The most common TEAE was injection site pain. Overall systemic exposure was comparable for both age groups. The mean (SD) attack rate during treatment decreased by 94.8% from baseline (1.84 [1.53] to 0.08 [0.17] attacks/mo), and 16 (76.2%) patients were attack-free. The attack rate reduction in both age groups was similar during the first 26-week fixed-dosing treatment. Seven patients switched from Q2W to Q4W and remained attack-free. A large, clinically meaningful increase in the Pediatric Quality of Life Inventory Generic Core Scale Total Score and a large increase in the PediatricQuality of Life Inventory Generic Core Scale-Family Impact Module Total Score from baseline to end of study (better HRQoL) were observed. Findings support safety, efficacy, and improved HRQoL with lanadelumab 150 mg Q2W and Q4W regimens for the prevention of HAE attacks in patients aged 2 to less than 12 years.

Tranexamic acid for angiotensin-converting enzyme inhibitor-induced angioedema.

Approximately 0.7% of patients taking angiotensin-converting enzyme inhibitors (ACEIs) develop ACEI-induced angioedema (ACEI-IA). With no approved treatments for ACEI-IA, the risk of complications is concerning. Tranexamic acid (TXA) has the potential to prevent intubations and resolve ACEI-IA by inhibiting the downstream production of bradykinin. In this review, we aim to evaluate the safety and efficacy of TXA use in ACEI-IA. We queried the PubMed database for studies involving TXA for ACEI-IA from January 2003 to January 2023. Seven studies met the study inclusion criteria. Our results demonstrate that TXA may improve angioedema symptoms and prevent intubation. In addition, its availability, low cost, and safety profile support its use for improving the symptoms and complications of ACEI-IA in an emergency setting.

Alteplase-induced facial orolingual angioedema (P12-5.012)

<h3>Objective:</h3> Represent post-thrombolysis angioedema, a relatively common adverse effect seen after administration of alteplase for acute ischemic stroke. <h3>Background:</h3> r-TPA induced angioedema is a common clinical finding after medication infusion for acute stroke. The incidence of this complication ranges from 1.3–8% (3, 4). Prognosis is usually good; most patients have symptom resolution within 72 hours (5). <h3>Design/Methods:</h3> Literature review revealed how signs and symptoms of angioedema differ in presentation and severity. We gathered case reports from the last decade to identify the most common location of stroke, site of edema, treatment used and complications of angioedema. <h3>Results:</h3> Symptoms usually began within 150 minutes after initial infusion (mean 70 minutes) and usually presented with unilateral angioedema, contralaterally to the side of the stroke. Approximately 30% of the patients required mechanical ventilation; most were on treatment with ACE inhibitors. The authors usually treated patients with a combination of steroids, antihistamines, adrenalin, or Icatibant, with complete recovery within 24 to 48 hours. <h3>Conclusions:</h3> Patients treated with Icatibant improved within minutes and achieved full remission in the first three hours. We believe it may become the standard of care in patients presenting with this uncommon but potentially life-threatening complication. <b>Disclosure:</b> Dr. Ayala Alvarez has nothing to disclose. Dr. Roque Sanchez has nothing to disclose. Dr. Jimenez-Ruiz has nothing to disclose. Dr. GOMEZ FIGUEROA has nothing to disclose. Omar Cardenas-Sanchez has nothing to disclose. Dr. Ruiz-Sandoval has nothing to disclose.

Safety and outcomes of “at-home self-provocation tests” in patients with mild nonsteroidal anti-inflammatory drug–induced urticaria/angioedema

BackgroundNonsteroidal anti-inflammatory drug (NSAID) hypersensitivity is common; however, many patients do not receive an accurate diagnosis and are using unnecessary alternative drugs or have medication restrictions. ObjectiveTo establish a protocol for provocation tests that can be performed safely and effectively at home to give patients an accurate diagnosis, whereas also delabeling NSAID hypersensitivity. MethodsWe retrospectively analyzed the medical records of 147 patients with NSAID hypersensitivity. All patients had NSAID-induced urticaria/angioedema with less than 10% body surface area skin involvement. One specialist developed the protocol through history taking and chart review. If NSAID hypersensitivity was confirmed, an oral provocation test was performed to confirm the safe alternative medications (group A). If it was undetermined, an oral provocation test was performed to confirm the diagnosis and alternative medications (group B). All oral provocation tests were performed by patients in their homes according to the protocol. ResultsApproximately 26% of group A patients had urticaria or angioedema symptoms with alternative drugs, whereas the remaining 74% was safe. In group B, 34% of the patients were diagnosed with having NSAID hypersensitivity. However, 61% did not respond to the culprit drug; therefore, NSAID hypersensitivity had been misdiagnosed. During this at-home self-provocation test, no severe hypersensitivity reactions occurred. ConclusionMany patients originally suspected of having NSAID hypersensitivity were confirmed to have been misdiagnosed. We successfully conducted an effective and safe at-home self-provocation test.

Applying bioinformatic analysis for prognostic assessment of the &lt;i&gt;HS3ST6&lt;/i&gt; missense mutations clinical significance in the development of hereditary angioedema

Hereditary angioedema (HAE) is a genetically determined disease characterized by recurrent attacks of edema affecting the subcutaneous and/or submucosal layers of tissue, face, lips, neck, extremities of the body, oral cavity, intestine and/or larynx. In the latter case, the disease becomes life-threatening. The majority of HAE cases are associated with decreased levels of C1 (C1-esterase inhibitor), there are also descriptions of HAE with dysfunctional C1 inhibitor and HAE with normal C1 inhibitor. In the first and second variants, mutations in the C1NH gene are the cause of the disease. HAE with normal quantitative and functional levels of C1-inhibitor has the same clinical manifestations but with mutations in other genes, including F12, PLG, ANGPT1, KNG1, MYOF, and HS3ST6. Currently, mutations in the HS3ST6 gene remain poorly understood; only one missense mutation (p.Thr144Ser, rs746467957) associated with the development of HAE has been described.The aim of our work was to study new mutations in the HS3ST6 gene and analyze in silico their prognostic nature and clinical significance for the development of hereditary angioedema.The material was whole blood samples obtained from 13 patients with symptoms of hereditary angioedema without reduced levels and function of C1-INH.Whole exome sequencing of patients, bioinformatic analysis of HS3ST6 gene mutations using a number of databases and Web resources to predict the effect of mutations on the protein and assess the conservatism of the positions of the mutations detected was involved in study methods.Mutations in the HS3ST6 gene were identified in four patients, including two cases with two mutations simultaneously. Application of bioinformatic analysis allowed us to obtain new data on four missense mutations in the studied gene. Potential pathogenetic significance was determined for three of them. The mutation NC_000016.9:g.1962132G&gt;A (p.A163V) is most likely to be involved in pathogenesis of HAE by indirect disruption of heparan sulfate O-sulfation directly within the protein. The NC_000016.9:g.1962024G&gt;A mutation (p.P199L) appears to lead to the development of the disease through disruption of docking with SDC2 heparan sulfate. In the NC_000016.9:g.1962046C&gt;T (p.A192T) mutation, destabilization of the 192 amino acid position next to PAPS, may contribute to disruption of heparan sulfate O-sulfation through disruption of protein functional activity and, therefore, catalysis transfer of sulfo group to heparan sulfate syndecan-2. Thus, in all three cases, the formation of HAE appears to be possible due to disruption of the O-sulfation steps of heparan sulfate syndecan-2.Considering that in silico methods offer new opportunities to assess the pathogenetic significance of mutations, the application of bioinformatic analysis can contribute to a detailed investigation of the causes of hereditary angioedema. The present work convincingly demonstrates that rare mutations in the HS3ST6 gene may be involved in the pathogenesis of HAE and provoke edema due to increased bradykinin release.

Efficacy of lanadelumab in angioedema due to acquired C1 inhibitor deficiency

Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease characterized by recurrent angioedema symptoms that are similar to those in the hereditary form of C1-INH deficiency (C1-INH-HAE).1 Symptom onset in C1-INH-AAE occurs after the 40 years of life in 90% of patients, and family history of angioedema is absent.2 Symptoms include recurrent cutaneous swellings; edema of the gastrointestinal mucosa causing temporary bowel obstruction with colicky abdominal pain, vomiting, and/or diarrhea; and life-threatening laryngeal edema.

Long term follow-up of complement parameters to improve the management of acquired angioedema due to C1-inhibitor deficiency

Acquired angioedema due to C1-inhibitor deficiency (C1–INH-AAE) is a rare disease that can be diagnosed via complement testing. It often accompanies lymphoproliferative underlying diseases. Our study aimed to examine if there is a connection between complement parameters and the clinical symptoms of C1–INH-AAE, and, in case of a known underlying disease, its activity. The other question is how a connection, if proven, could help in the development of the therapeutic strategy of C1–INH-AAE patients.In the past 30 years, out of the 3938 patients sent to the Angioedema Center with angioedema symptoms, we have diagnosed C1–INH-AAE in 19 cases. An underlying disease was diagnosed in 15 patients. Most often lymphoma (6/19 patients) and monoclonal gammopathy of undetermined significance (6/19 patients) were found.Angioedema specific long-term prophylaxis did not result in an improvement in neither the frequency of the attacks nor in the complement parameters.A connection has been found between the presence and activity of any underlying disease, the frequency of the angioedema attacks and the decreased level of proteins of the complement system. Decreasing complement parameters warn about the appearance or the worsening of the underlying disease. The treatment of the underlying disease brings improvement in the complement parameters.Rituximab treatment reduced the number of attacks or completely made them disappear, and we experienced positive changes in complement parameters. Complement parameters supported the long-term efficacy of rituximab treatment for C1–INH-AAE.The change in complement parameters predict the relapse of the underlying disease, and it is a good indicator for the prediction of angioedematous attacks. In C1–INH-AAE, it is essential to examine the patients for underlying diseases, and to regularly follow up the patient's complement parameters.

LIMITED CUTANEOUS SYSTEMIC SCLEROSIS MIMICKING HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR

<h3>Introduction</h3> Hereditary angioedema with normal C1 inhibitor (HAE-nl-C1INH) is often diagnosed when patients with antihistamine-resistant angioedema respond to bradykinin-targeted therapies. We present a patient with limited cutaneous scleroderma initially diagnosed as HAE-nl-C1INH, with variable response to HAE therapy. <h3>Case Description</h3> 56-year-old woman with hypertension presented with recurrent periorbital swelling while on ACE inhibitor therapy. After discontinuation, she continued to have episodes of angioedema for two months, responsive to recombinant C1 inhibitor therapy. Initial workup included normal C1 esterase inhibitor antigen and function, C4, C1q, SPEP, tryptase, and thyroid studies. After a six-month symptom-free period, angioedema recurred in the setting of stressful life events, viral infection, and antihypertensive medication changes. When on-demand therapy with recombinant C1 esterase inhibitor was observed to provide insufficient control, she started lanadelumab prophylaxis which stabilized her symptoms. She then developed episodic fluid retention with daily joint and subcutaneous swelling involving the hands, abdomen, and lower extremities coincident with additional changes to her antihypertensive regimen. Discontinuation of suspected culprit medications did not resolve the progression of her symptoms. Evaluation by rheumatology for joint symptoms and persistent hand swelling led to the diagnosis of limited cutaneous systemic sclerosis (scleroderma). Angioedema symptoms resolved after starting mycophenolate mofetil and she no longer required HAE therapy. <h3>Discussion</h3> Our case highlights the complicated and nuanced nature of diagnosing HAE-nl-C1INH, particularly in patients with partial response to HAE therapy. Limited cutaneous scleroderma either mimicked or triggered angioedema in our patient. Her partial response to C1 inhibitor infusions and lanadelumab may have represented off-target effects of HAE therapy.

Treatment of a Delayed Hypersensitivity-like Reaction after Second-line Therapy with Dabrafenib-Trametinib: A Case Report

Melanoma is the third most prevalent skin cancer with the highest mortality rate amongst skin cancers. Recent therapeutic developments have improved its treatment options, however significant toxicity can be a limiting factor. In this report, we describe a case of a rare, delayed hypersensitivity-like reaction after treatment with dabrafenib-trametinib as a second line therapy in a metastatic melanoma patient treated with pembrolizumab initially. A 62-year-old Caucasian male presented with a left axillary mass, which was found to be BRAF V600E positive malignant melanoma upon biopsy. He proceeded to have full axillary node dissection and was scheduled to receive adjuvant immunotherapy with pembrolizumab. However, the treatment was delayed by 3 weeks due to COVID-19. The patient developed subcutaneous metastases within that time. Although he was started on pembrolizumab, the metastases continued to progress. The patient then opted to begin dabrafenib-trametinib and was started on full dose therapy. After two weeks, there was significant reduction of metastases clinically. Two days after the follow-up, he presented to the emergency room with diffuse non-pruritic hives all over the body without any symptoms of angioedema. In addition, there was a sudden increase in size of the subcutaneous metastases. He was treated with 50 mg Benadryl and 50 mg prednisone with almost immediate resolution and discharged on prednisone. Dabrafenib-trametinib was temporarily discontinued. Due to the limited treatment options, the patient was carefully restarted on his dabrafenib-trametinib regimen with slow prednisone tapering. Patient has had an excellent response with complete resolution of his disease with no measurable lesions found on the latest CT scan. After a thorough literature search of OVID Medline + EMBASE, it was concluded that this is a rare presentation and only a few similar cases have been reported. Therefore, health care provider education and strategies for reintroduction of immunotherapy, as presented through a prednisone regimen in this case, is imperative for proper management of adverse events.

Diagnostics of chronic exercise-induced urticaria in a child (a case report)

This article shows a case report of exercise-induced urticaria diagnosis in an 8-year-old boy. The aim of this work is to show diagnostic peculiarities of chronic urticaria with known trigger. The necessity of diagnostic algorithm fully implementation is emphasized, even in presence of a clear correlation with trigger factor (in this case – physical exertion). The described case is interesting because previously healthy child without anamnesis of allergic and another chronic disease developed urticaria and symptoms of angioedema without visible cause. Objective examination did not reveal any clinically significant abnormalities except moderate nasal congestion, which was neglected by both the patient and his parents. His was without a past medical history of fever. The child underwent laboratory and instrumental examinations: clinical blood, urine and biochemical tests, immunological tests (cell-mediated and humoral immunity, phagocytosis). Acute phase response was within normal ranges. Total IgE was increased to 100.5 IU/ml, increased levels of specific IgE to cat allergen (41.13 кU/l), serum ß-lactoglobulin of cow’s milk (2.95 кU/l) and lamb meat (0.41 кU/l) were detected. Autoserum test was positive (histamine – papula of 5 mm, hyperemia of 15 mm, negative control – 0, patient’s serum – papula of 16.5 mm, hyperemia of 24 mm); thyroid autoantibodies and thyroid hormone levels were within normal ranges. Tests for physical urticarial were performed: aquagenic, cold, heat, vibratory were negative, dermographic urticaria was positive, test with physical exercise (treadmill) was positive (small-sized wheals and lower eyelid angioedema developed on the 14th minute of sweating). Detailing of anamnestic data revealed an appearance of small wheals after emotional sufferings, and a single episode of difficulty breathing with urticaria and angioedema symptoms while playing football). Conclusions. Thus, extended detailed diagnostics of this clinical case has revealed the combined influence of several factors (dermographic, physical exertion, sensitization to some allergens, autoimmune variant of urticaria/angioedema) clarifying that symptoms could be mediated by both IgE- and non-IgE-dependent mechanism in the same patient. Primary diagnosis of “chronic exercise-induced urticaria” has been changed to “anaphylaxis” requiring another approach to treatment and follow-up.

Late inflammatory reactions in patients with soft tissue fillers after SARS-CoV-2 infection and vaccination: A systematic review of the literature.

IntroductionSoft tissue fillers are used for cosmetic and reconstructive purposes, and soft tissue filler procedures are among the most common nonsurgical procedures in the USA. Although soft tissue filler procedures are relatively quick and safe, adverse events such as late inflammatory reactions have been reported with every filler product. Infections and vaccinations have been proposed as potential triggers for late inflammatory reactions (LIRs), and it is therefore not surprising that these adverse events have been reported after SARS‐CoV‐2 infection and vaccination. Therefore, this review aims to give a detailed overview of these cases.Materials and methodsA literature search was undertaken on LIRs in patients with a history of soft tissue filler use after SARS‐CoV‐2 infection or vaccination. This systematic review was reported according to the PRISMA guidelines. We searched the electronic database PubMed from January 2020 to August 2021. Data on patient characteristics, filler characteristics, clinical findings, and treatment options were included.ResultsThis review included 7 articles with a total of 19 patients with LIRs after SARS‐CoV‐2 infection or vaccination. Three patients with postinfection LIRs and 16 patients with postvaccination LIRs were reported. These LIRS mainly occurred in females who had HA injections for cosmetic purposes. Three patients with postinfection LIRs had symptoms of facial swelling and/or lip angioedema in a matter of weeks. Sixteen patients reported reactions after SARS‐CoV‐2 vaccination (13 following Moderna vaccination and 3 after Pfizer vaccination, after both the first and second doses) from 13 hours up to three weeks. These patients presented with similar clinical symptoms as patients with postinfection LIRs. All patients were treated in a conservative manner.DiscussionThis review shows a relationship between LIRs and SARS‐CoV‐2 infection and vaccination. In the case of vaccination, these adverse events have been reported only after Moderna and Pfizer vaccinations. The reported adverse events are generally minor and self‐limiting, and we encourage patients with soft tissue fillers to participate in vaccination programs.

HAE with normal C1-INH: Treatment and attack frequency based on data from the Canadian 2020 national survey

Hereditary angioedema with normal C1-esterase inhibitor (HAEnC1-INH) is associated with mutations in Factor XII and other genes. In the absence of genetic testing, diagnosis is based on clinical symptoms of angioedema and normal C1-INH functional levels so medication access can be difficult and available medications may not be fully effective.

COVID-19 triggers attacks in HAE patients without worsening disease outcome

COVID-19 triggers attacks in HAE patients without worsening disease outcome

Direct Amoxicillin Challenge Without Preliminary Skin Testing for Pediatric Patients With Penicillin Allergy Labels

To study the safety of direct provocation testing (DPT) without a preceding penicillin skin test (PST) in individuals considered low risk for immunoglobulin E (IgE)-mediated or non-IgE–mediated hypersensitivity on the basis of clinical history.A total of 54 pediatric (age: 1–17 years) patients at an academic-affiliated pediatric outpatient allergy clinic.This was a retrospective observational study. Patients were selected to undergo DPT without PST if considered low risk on the basis of clinical history. DPT was offered to patients with a history of nonspecific rash or urticaria. Patients were excluded if they had a history of (1) severe symptoms of respiratory distress, (2) vomiting, (3) angioedema, (4) mucosal involvement, (5) skin desquamation or blister formation, (6) target lesions, (7) new onset fever, (8) joint, kidney, or liver problems, and (9) required emergent treatment during the index reaction. A single dose of oral amoxicillin (20 mg/kg up to 500 mg) was given, and patients were observed for 60 minutes. Physical examination and vital signs were performed at baseline and every 15 minutes. If no reaction was noted, the penicillin allergy label was removed from the chart.A total of 54 patients previously labeled as penicillin allergic but considered low risk received DPT. One patient was excluded from analysis because of a diagnosis of anaphylaxis during the index reaction. The median age at index reaction was 2 years; the median age at DPT was 7 years. Nonspecific rash was reported by 57.4%, hives was reported by 38.9%, and emesis was reported by 5.5% during the index reaction. A total of 9.3% reported reaction within 1 hour of exposure, 42.6% reported reaction onset >1 hour after exposure, and 48.1% were unable to recall timing of index reaction. All 53 patients passed the DPT and had penicillin allergy removed from their chart. No adverse reactions were reported during the DPT, and there were no reports of delayed reactions.In this cohort of pediatric patients with penicillin allergy labels who were determined to be at low risk for immediate IgE-mediated hypersensitivity reactions, DPT without PST was found to be safe and effective in testing for penicillin allergy.It has been well established that the majority of patients labeled as penicillin allergic (up to 10% of US population) are not truly allergic. There has been a push to delabel patients, but this can involve several steps, including prick skin testing, intradermal testing, and an oral challenge. The time, cost, and invasiveness of this process may make this less attractive to children and their parents. This study supports other similar studies that, in low-risk patients, a direct oral challenge with observation is adequate for delabeling low-risk patients.

Angioedema induced by angiotensin-converting enzyme inhibitors: an analysis of hospitalizations during the COVID-19 pandemic

BACKGROUND: The pathogenesis of angioedema induced by angiotensin-converting enzyme inhibitors is based on the accumulation of bradykinin as a result of angiotensin-converting enzyme blockade. The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) binds to the angiotensin-converting enzyme 2 receptor, which may inhibit its production and thereby lead to an increase in bradykinin levels. Thus, SARS-CoV-2 infection may be a likely trigger for the development of angioedema.&#x0D; AIMS: This study aimed to analyze cases of hospitalizations of patients with angioedema associated with the use of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers during the coronavirus disease 2019 (COVID-19) pandemic.&#x0D; MATERIALS AND METHODS: This study retrospectively analyzed medical records of patients admitted to the Vitebsk Regional Clinical Hospital between May 2020 and December 2020 with isolated (without urticaria) angioedema while receiving angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. In all patients, smears from the naso- and oropharynx for COVID-19 were analyzed by polymerase chain reaction.&#x0D; RESULTS: Fifteen inpatients (9 men and 6 women) aged 4472 years were admitted because of emergent events, of which 53.6% had isolated angioedema. In two cases, a concomitant diagnosis of mild COVID-19 infection was established with predominant symptoms of angioedema, including edema localized in the face, tongue, sublingual area, and soft palate. All patients had favorable disease outcomes.&#x0D; CONCLUSIONS: Patients with аngiotensin-converting enzyme inhibitor-induced angioedema may require hospitalization to monitor upper respiratory tract patency. There were cases of a combination of аngiotensin-converting enzyme inhibitor-induced angioedema and mild COVID-19. Issues requiring additional research include the effect of SARS-CoV-2 infection on the levels of bradykinin and its metabolites, the triggering role of COVID-19 in the development of angioedema in patients receiving angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, recommendations for the management of patients with аngiotensin-converting enzyme inhibitor-induced angioedema, and a positive result for COVID-19.

Acquired Angioedema Due to C1 inhibitor Deficiency Caused by Non-Hodgkin Lymphoma in a Patient with Myasthenia Gravis

Abstract Acquired angioedema due to C1-inhibitor deficiency is a very rare disorder that usually appears in patients with lymphoproliferative and/or autoimmune diseases. This type of swelling is bradykinin mediated and does not respond to antihistamines, corticosteroids, or epinephrine. The symptoms usually appear in patients older than 40 years with recurrent episodes of angioedema without wheals. The family history is negative. The swelling could affect any tissue, but most frequently is located at the face, lips, tongue, larynx, or extremities. In the gastrointestinal tract, it causes pain, nausea, vomiting, and diarrhea. The upper respiratory airway oedema is a potentially life-threatening condition due to asphyxiation. The oedema attacks may precede the symptoms of the causative disease for months or years. In most cases, the treatment of the underlying disease resolves the angioedema episodes. Here we report a case of C1-INH-AAE caused by non-Hodgkin lymphoma in a patient diagnosed many years before with myasthenia gravis whose angioedema symptoms resolved after the specific treatment of lymphoma.

The Role of Infection and Autoimmunity in Urticaria and Angioedema as a Common Entity

Chronic spontaneous urticaria with angioedema is prevalent, affecting approximately 1% of the general population, and has a significant impact on quality of life, according to epidemiological data. This article aims to broaden the view on the mechanisms of urticaria and the role of infection in the current environment. It is not easy to identify the cause of urticaria but appropriate steps to treat an underlying infection can, in some cases, improve the symptoms of urticaria and angioedema, reduce severity and duration, or lead to remission. Although chronic spontaneous urticaria with angioedema is a multifactorial condition involving inflammation, autoimmunity, and coagulation, IgE-mediated autoimmunity, or autoallergy, is thought to play a major role. Every year, more is learnt about the role of cells releasing mediators, underlying autoimmune processes that lead to the development of mast cell activation and urticaria. It has become increasingly clear that mast cell roles in immune system responses are not limited to an allergic role; they are key players in protective immune responses, both innate and adaptive, to various pathogens and in defence of some infections. Several guidelines, consensus papers, and practice parameters have been developed for the management of chronic urticaria. The Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA2LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) produce a guideline, which is revised every 4 years by a global panel of experts in the field. Infections may be a cause, aggravating factor, or unassociated bystander in chronic urticaria. The author looked at evidence, using a keyword search, for the role of viral and bacterial infections in acute, acute recurrent, and chronic urticaria and angioedema, including COVID-19, herpes, viral hepatitis, and Helicobacter pylori.

Obstetric Anesthetic Management for Parturients with Hereditary Angioedema: A Case Report and Suggested Protocol

Abstract Hereditary angioedema (HAE) is a disease manifested by repeated episodes of localized submucosal or subcutaneous edematous episodes, potentially triggered by emotional stress, mechanical trauma, or intake of estrogens. We present our experience managing two parturients with HAE. Multidisciplinary care is essential for planning and executing the specialized care of these patients, and management included extensive planning among obstetric, anesthesiology, and allergy and immunology teams. Pregnancy has been shown to have a variable effect on triggering HAE episodes. First-line treatment includes C1 esterase inhibitor concentrate, which can also be used for prophylaxis in high-risk patients. Neuraxial analgesia is recommended to avoid general anesthesia and was established early in both individuals. Vaginal delivery was well tolerated without need for emergent treatment for angioedema symptoms.