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Exp Ther Med 2019 Feb 16;17(2):1068-1072. Epub 2018 Nov 16.

Allergology and Immunology Discipline, 'Iuliu Hațieganu' University of Medicine and Pharmacy, Cluj-Napoca 400012, Romania.

Angioedema can occur in isolation, accompanied by urticaria, or as a feature of anaphylaxis in mast cell-mediated disorders, bradykinin-mediated disorders, as well as in others with unknown mechanisms, such as infections, rare disorders, or idiopathic angioedema. In mast cell-mediated angioedema, other signs and symptoms of mast cell-mediator release are frequently seen. However, clear evidence of mast cell degranulation may be absent in histaminergic angioedema. Read More

Hautarzt 2019 Feb;70(2):101-106

Spezialsprechstunde Angioödeme, Hautklinik und Poliklinik, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland.

Manifestation of angioedema can occur at any age. We distinguish between two main subtypes: mast cell mediator-associated angioedema (often with hives) and the non-mast cell mediator-associated angioedema. The patient's history is very important due to the fact that one subtype can be hereditary, but we also have to consider new mutations and even not yet diagnosed patients. Read More

Allergy Asthma Clin Immunol 2019 5;15. Epub 2019 Jan 5.

2Department of Neuroscience, Division of Dentistry, University of Padua, Via Giustiniani 2, 35128 Padua, Italy.

Background: Melkersson-Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. Read More

Postepy Dermatol Alergol 2018 Dec 13;35(6):636-637. Epub 2018 Nov 13.

Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland.

MBio 2018 12 18;9(6). Epub 2018 Dec 18.

Department of Microbiology and Immunology, University of Michigan Medical School, Ann Arbor, Michigan, USA

Antibiotic-resistant is increasingly recognized as a cause of difficult-to-treat nosocomial infections, including pneumonia, wound infections, and bacteremia. Previous studies have demonstrated that the metalloprotease CpaA contributes to virulence and prolongs clotting time when added to human plasma as measured by the activated partial thromboplastin time (aPTT) assay. Here, we show that CpaA interferes with the intrinsic coagulation pathway, also called the contact activation system, in human as well as murine plasma, but has no discernible effect on the extrinsic pathway. Read More

Allergy 2018 Nov 29. Epub 2018 Nov 29.

Clinical Immunology and Allergy, Royal National Throat Nose and Ear Hospital, University College London Hospitals, London, UK.

J Am Acad Dermatol 2018 Nov 14. Epub 2018 Nov 14.

Loyola Univ Medical Center Dermatology, 321 N La Grange Rd, La Grange Park, IL. Electronic address:

Acquired cold urticaria (ACU) is characterized by the development of wheals, angioedema, or both after cold exposure. Age at diagnosis, disease severity, and response to the ice cube challenge test predicted both prognosis and response to therapy. Proper characterization of the type of acquired cold urticaria allows for appropriate counseling and choice of therapy. Read More

J Dermatolog Treat 2018 Nov 14:1-7. Epub 2018 Nov 14.

a 1 University Department Pro.Sa.M.I. "G. D'Alessandro", University of Palermo , Italy.

Chronic spontaneous urticaria is a clinical condition characterized by spontaneous or inducible recurrent wheals. This condition may significantly affect quality of life of patients and of their families. Aetiology is not identified in 25-85% of cases that are indicated as "idiopathic", because all diagnostic tests are negative. Read More

Presse Med 2018 Nov 8. Epub 2018 Nov 8.

Centre de référence national des angioedèmes (CREAK), 38043 Grenoble, France; Service d'immunologie, CHUGA, 38043 Grenoble, France.

Bradykinin mediated angioedema (BK-AE) can be associated either with C1Inhibitor deficiency (hereditary and acquired forms), either with normal C1Inh (hereditary form and drug induced AE as angiotensin converting enzyme inhibitors…). In case of high clinical suspicion of BK-AE, C1Inh exploration must be done at first: C1Inh function and antigenemy as well as C4 concentration. C1Inh deficiency is significant if the tests are below 50 % of the normal values and controlled a second time. Read More

Forensic Sci Med Pathol 2018 Nov 3. Epub 2018 Nov 3.

Forensic Science SA, 21 Divett Place, Adelaide, 5000, Australia.

An 86-year-old woman with a history of angioedema was found dead at her home address. She had recently complained of a swollen tongue. At autopsy the tongue was grossly edematous, protruding from the mouth. Read More

Allergy Asthma Clin Immunol 2018 25;14:69. Epub 2018 Oct 25.

1Carol Davila University of Medicine and Pharmacy, Bulevardul Eroii Sanitari, no. 8, District 5, 050474 Bucharest, Romania.

Background: Angioedema due to acquired deficiency of C1-inhibitor (C1-INH-AAE) is a rare disease sharing some clinical and laboratory similarities with hereditary angioedema, but with late onset and no positive family history. The underlining cause may be malignant or due to autoimmune diseases, but some cases remain idiopathic.

Case Presentation: We report a case of a 75 year old woman suffering from recurrent episodes of angioedema since the age of 66, considered first induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI). Read More

Int Arch Allergy Immunol 2019 2;178(1):50-59. Epub 2018 Oct 2.

Division of Allergy and Immunology, Department of Internal Medicine, Ege University Faculty of Medicine, Izmir, Turkey.

Background: How genotype affects phenotype in hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) has not been totally clarified. In this study, we investigated the relationship between different types of mutations and various phenotypic characteristics.

Methods: Clinical data from 81 patients from 47 families were recorded. Read More

Allergy Asthma Clin Immunol 2018 12;14(Suppl 2):59. Epub 2018 Sep 12.

3Section of Allergy & Clinical Immunology, Department of Internal Medicine, University of Manitoba, Winnipeg, MB Canada.

Urticaria (hives) is a common disorder that often presents with angioedema (swelling that occurs beneath the skin). It is generally classified as acute or chronic. Second-generation, non-sedating, non-impairing histamine type 1 (H1)-receptor antihistamines represent the mainstay of therapy for both acute and chronic urticaria. Read More

Trans Am Clin Climatol Assoc 2018 ;129:250-265

NEW YORK, NEW YORK.

Advances in understanding the immunological basis and mechanisms underlying allergic and immunologic disorders have led to effective but costly long-term and repetitive biologic therapies. Gene therapy is a rapidly advancing technology, in which a single administration of an adeno-associated virus encoding the therapeutic protein or monoclonal antibody may provide effective long-term therapy for allergic and immunologic disorders. In this review, we summarize the recent studies from our laboratory developing gene therapy strategies to treat hereditary angioedema and peanut allergy. Read More

Case Reports Immunol 2018 22;2018:8067610. Epub 2018 Jul 22.

Department of Biomedical Sciences, Humanitas University, Milan, Italy.

Presently, there is inconclusive evidence regarding the most effective treatment for idiopathic nonhistaminergic acquired angioedema (InH-AAE). Omalizumab may, however, prove to be a promising option. This case report describes two patients who presented with recurrent angioedema attacks, which was refractory to antihistamine therapy. Read More

Allergy Asthma Clin Immunol 2018 6;14:28. Epub 2018 Aug 6.

Department of Immunology, Central Manchester University Hospital NHS Foundation Trust, Manchester, UK.

Background: Hereditary angioedema (HAE) is a potentially life-threatening, bradykinin-mediated disease, often misdiagnosed and under-treated, with long diagnostic delays. There are limited real-world data on best-practice management of HAE in the UK.

Objectives: To characterize the clinical profile, management and outcomes of patients with HAE type I and II from three specialist centres in the UK using data from the Icatibant Outcome Survey (IOS; Shire, Zug, Switzerland), an international observational study monitoring safety and effectiveness of icatibant, a selective bradykinin B2 receptor antagonist. Read More

Med Oncol 2018 Aug 2;35(9):118. Epub 2018 Aug 2.

Department of Biomedical and Clinical Sciences Luigi Sacco, University of Milan, Luigi Sacco Hospital Milan, Milan, Italy.

Angioedema due to acquired deficiency of the inhibitor of the first component of complement (C1-INH) is a rare disease known as acquired angioedema (AAE). About 70% of patients with AEE display autoantibodies to C1-INH, the remaining patients have no antibodies to C1-INH. The clinical features of C1-INH deficiency include recurrent, self-limiting local swellings involving the skin, the gastrointestinal tract, and the upper respiratory tract. Read More

J Clin Lab Anal 2019 Jan 29;33(1):e22627. Epub 2018 Jul 29.

The Binding Site Group Limited, Birmingham, UK.

Background: Impaired levels or function of C1 inhibitor (C1-INH) results in angioedema due to increased bradykinin. It is important to distinguish between angioedema related to C1-INH deficiency and that caused by other mechanisms, as treatment options are different. In hereditary (HAE) and acquired (AAE) angioedema, C1-INH concentration is measured to aid patient diagnosis. Read More

J Allergy Clin Immunol Pract 2018 Jul - Aug;6(4):1132-1141

Department of Medicine, University of California, San Diego, and VA San Diego Healthcare System, San Diego, Calif. Electronic address:

Angioedema is an acute swelling of the deeper layers of the skin or mucosa resulting from a transient increase in vascular permeability. Angioedema can occur in the absence or presence of hives, be hereditary or acquired, and be caused by various potential mediators, including histamine and bradykinin. Bradykinin-mediated angioedema can be difficult to diagnose but is responsible for a disproportionate percentage of the serious morbidity and mortality associated with angioedema. Read More

J Am Board Fam Med 2018 Jul-Aug;31(4):650-652

From Jersey Shore University Medical Center, Neptune, NJ (ACK, MPC).

Galactose-α-1, 3 galactose (α-gal) is a carbohydrate found in mammalian meat. In 2007, it was implicated as a cause of severe hypersensitivity reactions when a study found elevated levels of antibodies directed against this oligosaccharide among patients treated with cetuximab, a monoclonal antibody that contained an α -gal epitope. The majority of these cases were reported in the Southeast United States in a distribution similar to that of Rocky Mountain spotted fever and ehrlichiosis, and that geographic association led researchers to the conclusion that a bite from the Lone Star tick can induce this antibody. Read More

Rheumatol Int 2018 Aug 27;38(8):1577-1584. Epub 2018 Jun 27.

Department of Pediatric of Rheumatology, Pamukkale University School of Medicine, Pamukkale, Denizli, Turkey.

An acquired form of angioedema that is clinically similar but scarcer than the hereditary form may be caused, even more rarely, by the presence of an underlying autoimmune disease. We report a previously healthy 16-year-old girl with an acquired angioedema as a rare and initial presentation of systemic lupus erythematosus. The patient had no previous angioedema attack and no family history. Read More

Allergy Asthma Clin Immunol 2018 4;14:24. Epub 2018 Jun 4.

Western Springs Asthma and Allergy, Western Spring, IL USA.

Background: Angioedema secondary to acquired C1 inhibitor deficiency (AAE) is a rare disease. It usually is associated with lymphoproliferative disorders. We present a case of AAE in a patient with antiphospholipid syndrome (APS), a non-Hodgkin lymphoproliferative disorder (NHL) with undetectable levels of C2, C4, and an undetectable CH50. Read More

Rev Med Interne 2018 Oct 4;39(10):772-776. Epub 2018 May 4.

Service de dermatologie, centre hospitalier Le Mans, 194, avenue Rubillard, 72037 Le Mans, France.

Introduction: Episodes of acquired bradykinin-mediated angioedema due to angiotensin-converting enzyme (ACE) inhibitors may result in fatal outcomes. There is no consensus regarding emergency pharmacological management of these episodes. Treatment options include icatibant and C1INH concentrate. Read More

Clin Chem Lab Med 2018 Aug;56(9):1498-1506

The Binding Site Group Limited, Birmingham, UK.

Background: The measurement of complement components is clinically useful where a deficiency is suspected, or where excessive activation and consumption are present in disease. C2 deficiency carries an increased risk of developing systemic lupus erythematosus, recurrent infections and atherosclerosis. In this study, we have evaluated The Binding Site's Human Complement C2 SPAPLUS® assay. Read More

Orphanet J Rare Dis 2018 May 4;13(1):73. Epub 2018 May 4.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Background: Bradykinin-mediated angioedema (Bk-AE) can be life-threatening and requires specific targeted therapies. Knowledge of its epidemiology may help optimize its management.

Methods: We systematically searched the medical literature to identify abstracts of interest indexed between 1948 and March, 2016. Read More

J Allergy Clin Immunol Pract 2018 Jul - Aug;6(4):1205-1208. Epub 2018 Apr 30.

Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary. Electronic address:

Background: The mechanism of idiopathic nonhistaminergic acquired angioedema (InH-AAE) has not yet been precisely elucidated. This condition is characterized by recurrent angioedema without wheals.

Objective: To study the clinical features of InH-AAE, and to make, for the first time, independent comparisons with hereditary angioedema of unknown origin (U-HAE), as well as with hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE). Read More

Ann Dermatol Venereol 2018 Oct 17;145(10):598-602. Epub 2018 Apr 17.

Dermatologie, CHRU Minjoz, 3, boulevard Alexandre-Fleming, 25030 Besançon, France.

Background: Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Read More

Cutis 2018 02;101(2):E14-E16

Department of Dermatology, Penn State Hershey Medical Center, USA.

Case Rep Hematol 2018 10;2018:7809535. Epub 2018 Jan 10.

Department of Medicine, Reading Hospital, West Reading, PA, USA.

Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. Read More

J Investig Allergol Clin Immunol 2018 ;28(1):62-64

Clínica Tambre, Madrid, Spain.

Anesth Pain Med 2017 Aug 22;7(4):e57801. Epub 2017 Jul 22.

Department of Anesthesiology and Perioperative Medicine, Drexel University College of Medicine, 245 N. 15th Street, Suite 7502, MS 310, Philadelphia, PA, 19102.

Introduction: An acquired Tracheoesophageal fistula (TEF) is commonly caused by a malignancy or trauma, with pulmonary infection or aspiration being the presenting symptom. However, in the critical care setting the presentation can be subtle and may present with difficult ventilation. High endotracheal tube cuff pressures can lead to tracheal erosions and thus increasing the chances for developing a TEF. Read More

Gastroenterology 2018 08 2;155(2):e11-e12. Epub 2018 Feb 2.

Department of Gastroenterology and Hepatology, University of Alabama at Birmingham, Alabama.

Int Arch Allergy Immunol 2018 26;175(3):126-135. Epub 2018 Jan 26.

Department of Medicine, Division of Allergy and Clinical Immunology, University of Salerno, Baronissi, Italy.

Recurrent angioedema (AE) without wheals is increasingly recognized as a clinical entity and a frequent cause of admission to the emergency room. The Hereditary Angioedema Working Group (HAWK) classification allowed the scientific community to go beyond the semantic confusion that dominated this topic for decades. This classification distinguishes hereditary and acquired forms of AE, either related or unrelated to C1 inhibitor deficiency. Read More

Sci Rep 2018 01 17;8(1):977. Epub 2018 Jan 17.

"L. Sacco" Department of Biomedical and Clinical Sciences, University of Milan, via GB Grassi 74, 20157, Milan, Italy.

C1-inhibitor is a serine protease inhibitor (serpin) controlling complement and contact system activation. Gene mutations result in reduced C1-inhibitor functional plasma level causing hereditary angioedema, a life-threatening disorder. Despite a stable defect, the clinical expression of hereditary angioedema is unpredictable, and the molecular mechanism underlying this variability remains undisclosed. Read More

Acute Med Surg 2017 10 18;4(4):462-466. Epub 2017 Aug 18.

Department of Emergency and Critical Care Medicine Japanese Red Cross Society Kyoto Daini Hospital Kyoto Japan.

Case: "Cannot intubate, cannot oxygenate" (CICO) is a rare, life-threatening situation. We describe a pediatric case of CICO and highlight some educational points.A 3-year-old boy who collapsed in the bathtub came to our emergency department. Read More

Pediatr Allergy Immunol 2017 12 24;28(8):850-852. Epub 2017 Oct 24.

Dermatology and Venereology, Medical University of Plovdiv, Plovdiv, Bulgaria.

Ann Allergy Asthma Immunol 2017 Oct 31;119(4):380-382. Epub 2017 Aug 31.

Division of Allergy and Clinical Immunology, Icahn School of Medicine, Mount Sinai, New York, New York.

Clin Transl Allergy 2017 31;7:27. Epub 2017 Aug 31.

Department of Clinical Immunology, University Hospital Zurich, Gloriastrasse 23, 8091 Zurich, Switzerland.

Background: Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease for which there are no available laboratory parameters to clearly define the disorder. Therapy is often difficult and various treatment options have been proposed. In this paper, we have evaluated the most effective therapies for InH-AAE on the basis of current literature and report the therapeutic effect of omalizumab in three patients with InH-AAE. Read More

Dtsch Arztebl Int 2017 Jul;114(29-30):489-496

Department of Otorhinolaryngology, Ulm University Hospital; Department of Anaesthesiology, Ulm University Hospital.

Background: Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated angioedema; the treatment of these two entities is fundamentally different.

Methods: This review is based on pertinent articles retrieved by a selective search in PubMed and on guidelines concerning the treatment of angioedema. Read More

Immunol Allergy Clin North Am 2017 08 15;37(3):497-511. Epub 2017 May 15.

Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Cox 201 Allergy Associates, Boston, MA 02114, USA.

Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH. A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE. The most common conditions associated with C1-INH-AAE are autoimmunity and B-cell lymphoproliferative disorders. Read More

SAGE Open Med Case Rep 2017 8;5:2050313X17713912. Epub 2017 Jun 8.

Department of Anesthesiology and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

Objective: To describe the perioperative management of a patient with acquired angioedema (AAE).

Methods: A 66-year-old Caucasian male presented from an outside hospital with a history of acquired angioedema and gastrointestinal stromal tumor-related intractable urticaria and mastocytosis. He was admitted for urgent laparoscopic partial gastrectomy, secondary to gastric outlet obstruction symptomatology. Read More

Allergy Asthma Clin Immunol 2017 13;13:28. Epub 2017 Jun 13.

Division of Allergy and Clinical Immunology, Department of Medicine, St. Michael's Hospital, Toronto, Canada.

Rationale: Angioedema without co-existent urticaria is due to a limited number of causes, including hereditary and acquired C1 esterase inhibitor deficiency, drug-induced angioedema or idiopathic histaminergic or non-histaminergic angioedema. We describe a cohort of patients with recurrent angioedema whose clinical features and response to medications are distinct from the causes above.

Methods: Patients were accrued retrospectively from an academic allergy practice between 2007 and 2014. Read More

Int J Emerg Med 2017 Dec 13;10(1):15. Epub 2017 Apr 13.

Department of Emergency Medicine, University Hospital, Aintree, Liverpool, UK.

Background: Angioedema is a common presentation in the emergency department (ED). Airway angioedema can be fatal; therefore, prompt diagnosis and correct treatment are vital.

Objective Of The Review: Based on the findings of two expert panels attended by international experts in angioedema and emergency medicine, this review aims to provide practical guidance on the diagnosis, differentiation, and management of histamine- and bradykinin-mediated angioedema in the ED. Read More

Dtsch Arztebl Int 2017 03;114(10):178

J Stomatol Oral Maxillofac Surg 2017 Apr 22;118(2):109-114. Epub 2017 Feb 22.

Service d'odontologie, hôpital Saint-Julien, CHU de Rouen, 76031 Rouen, France.

Bradykinin-mediated angioedema (AE) is a rare disease characterized by recurrent cutaneous or mucosal angioedema. This hereditary or acquired disease is of rapid installation, non-pruritic, usually painless and can affect the face, lips, larynx, gastrointestinal tract or extremities. When the affected area involves the upper respiratory tract, laryngeal angioedema can lead to imminent death by asphyxia. Read More

Clin Imaging 2017 May - Jun;43:122-126. Epub 2017 Mar 8.

Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N. Saint Clair St., Suite 800, Chicago, IL 60611, United States. Electronic address:

Angioedema is a condition in which an increase in vascular permeability leads to the swelling of body tissues. There are both hereditary and acquired forms of the disease, with the latter often associated with the administration of angiotensin-converting enzyme inhibitor medication. Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. Read More

Crit Care Med 2017 Apr;45(4):725-735

1Division of Pulmonary, Critical Care, Allergy and Immunology, Department of Medicine, Wake Forest Baptist Medical Center, Winston-Salem, NC.2Division of Allergy and Clinical Immunology, Department of Medicine, W.G. (Bill) Hefner VA Medical Center, Salisbury, NC.

Objectives: Angioedema is a potentially life-threatening occurrence that is encountered by critical care providers. The mechanistic understanding of angioedema syndromes has improved in recent years, and novel medications are available that improve outcomes from these syndromes. This clinically focused review will describe the underlying genetics, pathophysiology, classification and treatment of angioedema syndromes, with an emphasis on the novel pharmacologic agents that have recently become available for acute treatment. Read More

J Allergy Clin Immunol Pract 2017 Sep - Oct;5(5):1307-1313. Epub 2017 Mar 9.

Department of Biomedical and Clinical Sciences, Luigi Sacco Hospital, University of Milan, Milan, Italy; ASST Fatebenefratelli Sacco, Milan, Italy.

Background: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease with no prevalence data or approved therapies.

Objective: To report data on patients with C1-INH-AAE followed at Angioedema Center, Milan (from 1976 to 2015).

Methods: Diagnostic criteria included history of recurrent angioedema without wheals; decreased C1-INH antigen levels and/or functional activity of C1-INH and C4 antigen less than 50% of normal; late symptom onset (>40 years); no family history of angioedema and C1-INH deficiency. Read More