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Arthritis Care Res (Hoboken) 2021 May 31. Epub 2021 May 31.

Clinical Immunology, Angioedema and Allergy Unit, The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Tel Aviv, Israel.

Background: The Antiphospholipid Syndrome (APS) is an acquired coagulopathy associated with the presence of antiphospholipid antibodies. Whether ethnicity modulates APS clinical course is not known. The aim of our study was to assess the interplay ethnicity and APS in Israel. Read More

Actas Dermosifiliogr 2021 May 28. Epub 2021 May 28.

Immunoallergology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal.

Introduction: Cold Urticaria (ColdU) is a type of chronic inducible urticaria (CIndU) where recurrent pruritic wheals and/or angioedema occur after exposure to cold stimulus. Although it usually only affects exposed areas, systemic reactions can occur in severe cases. In this study, we seek to characterize the ColdU cases within our Centre's population of patients. Read More

J Allergy Clin Immunol Pract 2021 May 20. Epub 2021 May 20.

Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany. Electronic address:

Background: Lanadelumab has been available in Germany for the prophylactic treatment of hereditary angioedema since February 2019.

Objective: To investigate real-life treatment outcome of lanadelumab and gain practical experience in adapting the therapy to individual patients.

Methods: The study included 34 patients. Read More

Ned Tijdschr Tandheelkd 2021 May;128(5):269-276

Angioedema stems from increased vasodilation and vascular permeability, resulting in extravasation of fluid. Hereditary and acquired types of angioedema can be distinguished, with 3 and 4 subtypes, respectively. Groups of medicaments potentially inducing angioedema are, among others: ACE inhibitors, angiotensin II receptor blockers, dipeptidyl peptidase-4 inhibitors, thrombocyte aggregation inhibitors and immunosuppressive agents. Read More

J Clin Med 2021 Apr 1;10(7). Epub 2021 Apr 1.

Department of Translational Medical Sciences and Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Via S. Pansini 5, 80131 Naples, Italy.

Episodic angioedema with eosinophilia (EAE) (Gleich's syndrome) is a rare disease characterized by hypereosinophilia (up to 95 × 10 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3-4 weeks). The exact etiology of EAE is still unclear, but both eosinophils and abnormalities of cytokines homeostasis seem to play a pivotal role in the pathogenesis of the disease. In particular, the cyclic elevation of serum interleukin-5 before the increase in eosinophil count has been reported. Read More

Actas Dermosifiliogr 2021 Apr 24. Epub 2021 Apr 24.

Immunoallergology Department, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte, Lisboa, Portugal.

Introduction: Cold Urticaria (ColdU) is a type of chronic inducible urticaria (CIndU) where recurrent pruritic wheals and/or angioedema occur after exposure to cold stimulus. Although it usually only affects exposed areas, systemic reactions can occur in severe cases. In this study, we seek to characterize the ColdU cases within our Centre's population of patients. Read More

Clin Res Hepatol Gastroenterol 2021 Apr 14:101702. Epub 2021 Apr 14.

Director, Division of Gastroenterology and Liver Disease, The George Washington University, 3131 I St NW, Washington, DC, United States.

Angioedema can be either mast cell- (histamine-) mediated or bradykinin-mediated. Treatment approaches for the two types are very different, making differential diagnosis critical. Severe acute abdominal pain caused by intestinal angioedema is commonly misdiagnosed, especially when associated with bradykinin-mediated angioedema. Read More

Einstein (Sao Paulo) 2021 9;19:eRW5498. Epub 2021 Apr 9.

Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.

Angioedema attacks are common causes of emergency care, and due to the potential for severity, it is important that professionals who work in these services know their causes and management. The mechanisms involved in angioedema without urticaria may be histamine- or bradykinin-mediated. The most common causes of histamine-mediated angioedema are foods, medications, insect sting and idiopathic. Read More

J Allergy Clin Immunol Pract 2021 Jun 2;9(6):2186-2194. Epub 2021 Apr 2.

Department of Pediatrics, Federal University of São Paulo, São Paulo, Brazil.

Angioedema and urticaria affect people of all ages. Accurate diagnosis and optimum management is essential for healthy aging. Older people continue to experience mast cell-mediated urticaria and angioedema, with a higher prevalence of autoimmune and a lower prevalence of autoallergic disease. Read More

Int J Mol Sci 2021 Mar 9;22(5). Epub 2021 Mar 9.

Synapse Research Institute, 6217 KD Maastricht, The Netherlands.

Fibrinolysis is an important process in hemostasis responsible for dissolving the clot during wound healing. Plasmin is a central enzyme in this process via its capacity to cleave fibrin. The kinetics of plasmin generation (PG) and inhibition during fibrinolysis have been poorly understood until the recent development of assays to quantify these metrics. Read More

Clin Rev Allergy Immunol 2021 Mar 3. Epub 2021 Mar 3.

Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.

The Angioedema Quality of Life Questionnaire (AE-QoL) is an angioedema (AE)-specific validated questionnaire, which surveys the quality of life of diagnosed patients. The questionnaire has been used in multiple clinical trials. Our aim was to investigate how the questionnaire can assist physicians in the everyday practice of following up and managing C1-inhibitor deficiency patients. Read More

JAAD Case Rep 2021 Mar 17;9:52-53. Epub 2021 Jan 17.

Division of Allergology, University Children's Hospital Zurich, Zurich, Switzerland.

Balkan Med J 2021 Mar;38(2):73-81

Division of Immunology-Allergy, Department of Internal Medicine, Ege University School of Medicine, İzmir, Turkey.

Angioedema without wheals (urticaria) represents a heterogeneous group of clinically indistinguishable diseases of hereditary or acquired etiology. Hereditary angioedema is a rare inherited condition leading to recurrent, sometimes life-threatening angioedema attacks in subcutaneous tissues and gastrointestinal and oropharyngeal mucosa dating back to childhood or adolescence. Most of these patients have mutations in the SERPING1 gene, causing either low C1 inhibitor production (hereditary angioedema with C1 inhibitor deficiency type I) or the production of dysfunctional C1 inhibitor (hereditary angioedema with C1 inhibitor deficiency type II). Read More

Allergy Asthma Clin Immunol 2021 Feb 15;17(1):18. Epub 2021 Feb 15.

Allergy and Immunology, University of Manitoba, Winnipeg, Canada.

Introduction: Angiotensin Converting Enzyme Inhibitors (ACEI) are a common cause of Emergency Room presentation for angioedema. Although no treatment guidelines exist, C1 esterase inhibitor concentrate (C1-INH) is used on an off label basis for management of ACEI acquired angioedema (ACEI AAE).

Objective: To evaluate the efficacy of C1-INH in management of ACEI AAE at our local centers. Read More

J Allergy Clin Immunol Pract 2021 Jun 6;9(6):2490-2491. Epub 2021 Feb 6.

Department of Internal Medicine, Grenoble University Hospital, Grenoble, France; National Reference Centre for Angioedema, CREAK, Grenoble, France.

Clin Rev Allergy Immunol 2021 Jan 28. Epub 2021 Jan 28.

Department of Medicine, Division of Rheumatology, Allergy and Immunology, University of California San Diego, San Diego, CA, USA.

Biochemical studies performed during the last decades resulted in the development of various innovative medicinal products for hereditary angioedema (HAE). These therapeutic agents target the production or the function of bradykinin-the main mediator of HAE due to C1-inhibitor (C1-INH) deficiency. However, despite these remarkable achievements, current knowledge cannot provide convincing explanations for the clinical variability of the disease. Read More

Int Arch Allergy Immunol 2021 Jan 20:1-8. Epub 2021 Jan 20.

Department of Immunology, 2nd Faculty of Medicine Charles University and Motol University Hospital, Prague, Czechia.

Introduction: Acquired angioedema with C1 inhibitor deficiency (AAE-C1-INH) is rare but a potentially life-threatening disease. There are no official prevalence data, nor approved therapies for this condition.

Objective: In this study, we aimed to collect and analyze clinical data on patients with AAE-C1-INH in the Czech Republic. Read More

J Allergy Clin Immunol Pract 2021 Jun 2;9(6):2293-2304.e5. Epub 2020 Dec 2.

Department of Biophysics, Federal University of São Paulo, São Paulo, Brazil.

Background: Primary angioedema (PA) is a complex disorder, presenting multiple hereditary (hereditary angioedema) and acquired subtypes (acquired angioedema). Despite a very similar clinical presentation among subtypes, the differential diagnosis is limited by the difficulty to identify bradykinin-mediated PA and the lack of specific biomarkers.

Objectives: To report the clinical and genetic features of Brazilian patients with PA. Read More

J Emerg Med 2021 Jan 17;60(1):35-43. Epub 2020 Nov 17.

Intermountain Healthcare, Provo, Utah.

Background: Angioedema (AE) is a clinical syndrome marked by localized swelling of the subcutaneous layer of the skin or the submucosal layer of the respiratory or gastrointestinal tracts. While AE is commonly mediated by histamine (allergic AE), some types result from excessive bradykinin activity, including hereditary AE (HAE), acquired AE, and angiotensin-converting enzyme inhibitor-induced AE. These are less common but important to consider given different treatment requirements and potentially serious outcomes, including death from laryngeal swelling. Read More

J Intern Med 2021 Apr 20;289(4):547-558. Epub 2020 Nov 20.

From the, Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.

Background: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare form of bradykinin-mediated angioedema. It is diagnosed by complement testing; its treatment consists of the management of angioedema (AE) attacks and of underlying disease.

Objective: Evaluate the results of the clinical follow-up of patients with C1-INH-AAE. Read More

Anaesth Rep 2020 Jul-Dec;8(2):131-134. Epub 2020 Oct 16.

Department of Anaesthesiology Rotunda Hospital Dublin 1 Ireland.

A 35-year-old gravida 3, para 0 woman required an emergency caesarean section for pre-eclampsia with severe features including a platelet count of 7 × 10.l. The patient's thrombocytopaenia was an acute on chronic presentation. Read More

J Clin Med 2020 Oct 23;9(11). Epub 2020 Oct 23.

CeMIA SA, Makriyianni 31, GR-41334 Larissa, Greece.

The genetic alteration underlying the great majority of primary angioedema with normal C1 inhibitor (nl-C1-INH-HAE) cases remains unknown. To search for variants associated with nl-C1-INH-HAE, we genotyped 133 unrelated nl-C1-INH-HAE patients using a custom next-generation sequencing platform targeting 55 genes possibly involved in angioedema pathogenesis. Patients already diagnosed with alterations as well as those with histaminergic acquired angioedema were excluded. Read More

Allergy Asthma Proc 2020 11;41(Suppl 1):S03-S07

Division of Immunology/Allergy Section, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio; and.

Hereditary angioedema (HAE) is defined as a rare genetic disease with recurrent episodes of localized bradykinin-mediated swelling of the deep tissues of the skin, respiratory, and gastrointestinal tracts that can be life threatening. Classification of HAE has evolved over time with our further understanding of clinical phenotypes, underlying causes, and available testing. In most cases, HAE is caused by a deficiency of C1-esterase inhibitor (C1-INH) on the Serpin Family G Member 1 (SERPING1) gene, either through decreased amounts of C1-INH protein (C1-INH-HAE, type 1) or decreased function of C1-INH (C1-INH-HAE, type 2). Read More

Int Rev Immunol 2021 16;40(3):159-170. Epub 2020 Oct 16.

Department of Translational Medical Sciences - Section of Pediatrics, Federico II University of Naples, Naples, Italy.

The complement system is a multi-functional system representing the first line host defense against pathogens in innate immune response, through three different pathways. Impairment of its function, consisting in deficiency or excessive deregulated activation, may lead to severe systemic infections or autoimmune disorders. These diseases may be inherited or acquired. Read More

Postepy Dermatol Alergol 2020 Aug 2;37(4):445-451. Epub 2020 Sep 2.

Department of Dermatology, Venerology and Allergology, Medical University of Gdansk, Gdansk, Poland.

Angioedema is a non-inflammatory oedema of the subcutaneous tissue and/or mucosal membranes. It most commonly coexists with urticaria wheals and is considered to be a deep form of urticaria. Less commonly, it occurs in isolation and can take two basic forms: acquired angioedema and hereditary angioedema. Read More

J Allergy Clin Immunol Pract 2021 02 19;9(2):971-984. Epub 2020 Sep 19.

Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Berlin, Germany. Electronic address:

Background: Vibratory angioedema (VA) is a subtype of chronic inducible urticaria that manifests with erythematous wheals or angioedema after skin exposure to vibration. Because the condition is rare, the available information is limited.

Objective: To systematically review the clinical manifestations and treatment options of VA. Read More

J Allergy Clin Immunol Pract 2021 02 8;9(2):947-955. Epub 2020 Sep 8.

Hungarian Angioedema Reference Center, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary. Electronic address:

Background: Angiotensin-converting enzyme inhibitors may cause angioedema. Currently, no laboratory method is available for identifying acquired angioedema related to angiotensin-converting enzyme inhibitors. However, establishing the diagnosis is possible from the medical history and the preexisting angiotensin-converting enzyme inhibitor therapy, as well as by excluding other angioedema types. Read More

Int Arch Allergy Immunol 2020 7;181(12):941-946. Epub 2020 Sep 7.

Department of Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil,

Background: Acquired angioedema due to C1 inhibitor deficiency (AAE-C1-INH) is a very rare disease. In clinical practice, it may be difficult to differentiate AAE-C1-INH from hereditary angioedema due to C1-INH deficiency (HAE-C1-INH). In both conditions, patients are at an increased risk of death from asphyxiation due to upper airway obstruction. Read More

Rev Alerg Mex 2020 Apr-Jun;67(2):96-101

Benemérita Universidad Autónoma de Puebla, Facultad de Medicina, Puebla, México.

Background: Trimethoprim-sulfamethoxazole (TMP-SMZ) is the long-term use antimicrobial of choice in the prevention and treatment of opportunistic germs in patients with acquired immunodeficiency syndrome (AIDS) in whom the frequency of ADR (adverse drug reactions) is of 30% to 50 %.

Objective: To determine the adverse reactions to TMP-SMZ and their risk factors in AIDS patients.

Methods: The patients included in the study were older than 18 years of age, admitted from January 2018 to May 2019 with a confirmed diagnosis of HIV, and had had adverse drug reactions; 319 files were reviewed. Read More

Leuk Lymphoma 2020 12 25;61(13):3280-3281. Epub 2020 Aug 25.

Department of Medicine, Division of Hematology and Oncology, University of Illinois at Chicago, Chicago, IL, USA.