Immunol Allergy Clin North Am 2017 Aug 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 Mar;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 Mar 8. Epub 2017 Mar 8.

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

Int Arch Allergy Immunol 2017 22;172(1):55-63. Epub 2017 Feb 22.

Rheumatology, Allergology and Clinical Immunology, Department of "Medicina dei Sistemi", University of Rome Tor Vergata, Rome, Italy.

Background: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. Read More

G Ital Cardiol (Rome) 2016 Dec;17(12):966-972

Dipartimento di Scienze Biomediche e Cliniche "Luigi Sacco", Università degli Studi, Ospedale Luigi Sacco, Milano.

The definition of angioedema is an edema of subcutaneous and submucosal tissues due to increased vascular permeability and fluid extravasation. It can affect different areas, including extremities, genitals, upper airways and intestinal mucosa. The symptoms are disabling and this condition can be fatal if it involves the larynx. Read More

BMJ Case Rep 2017 Jan 10;2017. Epub 2017 Jan 10.

Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.

Angioedema (AE) of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to increased use of medications inhibiting the degradation of vasoactive peptides. Acquired angioedema related to angiotensin-converting enzyme inhibitors (ACEI-AAE) is well known, but other pharmaceutical agents also affect the degradation of bradykinin and substance P. Read More

Clin Exp Immunol 2017 Apr 9;188(1):148-153. Epub 2017 Feb 9.

Shire, Zug, Switzerland.

Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE types I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrolment. Read More

Immunol Allergy Clin North Am 2017 Feb 28;37(1):201-215. Epub 2016 Oct 28.

Yale School of Medicine, Gesher LLC, Allergy, Asthma and Clinical Immunology, 4 Clifton Avenue, Waterbury CT 06710, USA. Electronic address:

Differential diagnosis of urticaria and angioedema has been based on the phenotype as either acute or chronic depending on the duration of more than 6 to 8 weeks, respectively. Additional subdivisions include poorly defined terms such as idiopathic, spontaneous, or autoimmune. In this article, the author suggests that an increased understanding of the acquired and innate immune system and data from novel proteomic technology have blurred the lines between these categories of diagnosis. Read More

J Clin Immunol 2017 Jan 8;37(1):80-84. Epub 2016 Nov 8.

Internal Medicine Department, Grenoble University Hospital, Grenoble, France.

Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease characterized by AE resistant to antihistamines and a chronic course. We report five new cases of InH-AAE (two women and three men) with a rapid and dramatic response to the anti-immunoglobulin-E antibody omalizumab. In our literature review, we found 13 other relevant cases with a good response to this treatment. Read More

Rev Alerg Mex 2016 Oct-Dec;63(4):373-384

Universidad de Antioquia. Medellín, Antioquia, Colombia.

Angioedema is defined as edema of the skin or mucosa, including the respiratory and the gastrointestinal mucosa, which is self-limiting, and in most cases is completely resolved in less than 72 hours. It occurs due to increased permeability of the mucosal and submucosal capillaries and postcapillary venules, with resulting plasma extravasation. There are different types of angioedema: histaminergic (which may be mediated by immunoglobulin E), hereditary, from acquired C1 inhibitor deficiency, from angiotensin converting enzyme inhibitor, bradykinin-mediated, and non-histaminergic idiopathic angioedema. Read More

Adv Clin Chem 2016;77:1-75. Epub 2016 Jul 5.

Mayo Clinic, Rochester, MN, United States. Electronic address:

Historically, complement disorders have been attributed to immunodeficiency associated with severe or frequent infection. More recently, however, complement has been recognized for its role in inflammation, autoimmune disorders, and vision loss. This paradigm shift requires a fundamental change in how complement testing is performed and interpreted. Read More

J Clin Anesth 2016 Nov 5;34:385-91. Epub 2016 Jun 5.

Department of Anesthesiology, Mayo Clinic, Rochester, MN. Electronic address:

Purpose: Two types of bradykinin-mediated angioedema, hereditary angioedema (HAE) and acquired angioedema (AAE), result from deficiency or dysfunction of C1 esterase inhibitor, leading to an overproduction of bradykinin, which can lead to vascular permeability and life-threatening angioedema of the airway. The objective of this study was to review perioperative outcomes in a series of patients with HAE and AAE and to review current knowledge about anesthetic complications in patients with HAE or AAE.

Methods: Medical records were retrospectively reviewed for perioperative complications in patients with HAE or AAE who underwent general anesthesia from January 1, 2000, to December 31, 2014, at our institution. Read More

Medicine (Baltimore) 2016 Aug;95(33):e4363

aInternal Medicine Department, Saint Antoine Hospital, Assistance Publique-Hôpitaux de Paris, DHU i2B, Paris 6 University, Paris bHematology Department, Pitié Salpétrière Hospital, Assistance Publique-Hôpitaux de Paris, Paris 6 University, Paris cImmunology Laboratory, University Hospital, Grenoble dCentre de Référence et d'Etude des Angioedèmes à Kinine (CREAK) , Grenoble ePublic Health Department, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, Paris 6 University fImmunology Laboratory, Georges Pompidou European Hospital, Assistance Publique-Hôpitaux de Paris, Paris 5 University, Paris gJoint Unit 1036 CNRS-CEA-INSERM, University Grenoble Alpes hInternal Medicine Department, University Hospital, Grenoble iUniversité Joseph Fourier Grenoble, GREPI/AGIM CNRS FRE 3405, Grenoble jInternal Medicine Department, La Conception Hospital, AP-HM, Marseille kInternal Medicine and Clinical Immunology Department, Lille University Hospital lLIRIC, INSERM UMR 995, EA2686, Lille mDermatology Department, L'UNAM Université, University Hospital, Angers, France.

Acquired angioedema (AAE) due to C1-inhibitor (C1INH) deficiency is rare. Treatment options for acute attacks are variable and used off-label. Successful treatment of the associated lymphoma with rituximab seems to prevent acute attacks in subjects with AAE. Read More

J Eur Acad Dermatol Venereol 2017 Jan 8;31(1):45-52. Epub 2016 Aug 8.

Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Read More

J Am Acad Dermatol 2016 Nov 30;75(5):918-924.e2. Epub 2016 Jul 30.

Department of Dermatology, Hospital del Mar- Institut Mar d'Investigacions Mèdiques, Universitat Autònoma de Barcelona (UAB), Barcelona, Spain.

Background: Data about special phenotypes, natural course, and prognostic variables of patients with acquired cold urticaria (ACU) are scarce.

Objectives: We sought to describe the clinical features and disease course of patients with ACU, with special attention paid to particular phenotypes, and to examine possible parameters that could predict the evolution of the disease.

Methods: This study was a retrospective chart review of 74 patients with ACU who visited a tertiary referral center of urticaria between 2005 and 2015. Read More

J Investig Allergol Clin Immunol 2016 ;26(4):212-21; quiz two pages after page 221

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

Angioedema is defined as local, noninflammatory, self-limiting edema that is circumscribed owing to increased leakage of plasma from the capillaries located in the deep layers of the skin and the mucosae. Two mediators, histamine and bradykinin, account for most cases of angioedema. Angioedema can occur with wheals as a manifestation of urticaria, and this form is frequently allergic. Read More

Int Arch Allergy Immunol 2016 28;170(2):101-7. Epub 2016 Jul 28.

Department of Dermatology, Johannes Gutenberg University, Mainz, Germany.

Objective: To evaluate whether activated partial thromboplastin time (APTT) could be used in the laboratory diagnosis of hereditary or acquired angioedema (HAE or AAE) with and without C1 inhibitor (C1-INH) deficiency.

Methods: In a prospective investigation, APTT and other coagulation parameters were determined in 149 adult patients with various types of angioedema and in 26 healthy participants (HP).

Results: Mean APTT was significantly shortened in HAE-C1-INH type I (p < 0. Read More

Dimens Crit Care Nurs 2016 Jul-Aug;35(4):181-9

Angela Smith Collins-Yoder PhD, RN, CCNS, ACNS BC, is a clinical professor at the University of Alabama Capstone College of Nursing and is a critical care clinical nurse specialist.

Angioedema (AE) is a unique clinical presentation of an unchecked release of bradykinin. The origin of this clinical presentation can be either genetic or acquired. The outcome within the patient is subcutaneous swelling of the lower layers of the epidermis. Read More

R I Med J (2013) 2016 Jun 1;99(6):41-4. Epub 2016 Jun 1.

Clinical Associate Professor of Medicine, Alpert Medical School of Brown University.

Hereditary and acquired angioedema are potentially life-threatening diseases characterized by spontaneous episodes of subcutaneous and submucosal swelling of face, lips, oral cavity, larynx, and GI tract. Hereditary angioedema (HAE) usually presents within the first and second decades of life, whereas acquired angioedema presents in adults after 40 years of age. These clinical symptoms together with reduced C1 inhibitor levels and/or activity can usually confirm the diagnosis. Read More

J Investig Allergol Clin Immunol 2016 ;26(2):111-2

Department of Medicine, University of Salerno, Salerno, Italy.

Clin Rev Allergy Immunol 2016 Oct;51(2):140-51

Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4, H-1125, Budapest, Hungary.

Angioedema, as a distinct disease entity, often becomes a clinical challenge for physicians, because it may cause a life-threatening condition, whereas prompt and accurate laboratory diagnostics may not be available. Although the bedside diagnosis needs to be established based on clinical symptoms and signs, family history, and the therapeutic response, later, laboratory tests are available. Currently, only for five out of the nine different types of angioedema can be diagnosed by laboratory testing, and these occur only in a minority of the patient population. Read More

Case Rep Emerg Med 2016 31;2016:3930923. Epub 2016 Mar 31.

Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen East, Denmark.

Angioedema of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to pharmaceuticals influencing the generation or degradation of the vasoactive molecule bradykinin. Plasma-derived C1-esterase inhibitor concentrate is a well-established treatment option of hereditary and acquired complement C1-esterase inhibitor deficiency, which are also mediated by an increased level of bradykinin resulting in recurrent angioedema. Read More

Clin Rev Allergy Immunol 2016 Oct;51(2):162-9

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

Due to marked heterogeneity of clinical presentations, comprehensive knowledge of angioedema phenotypes is crucial for correct diagnosis and choosing the appropriate therapeutic approach. One of the ways to a meaningful clinical distinction can be made between forms of angioedema occurring "with or without wheals." Angioedema with wheals (rash) is a hallmark of urticaria, either acute or chronic, spontaneous or inducible. Read More

Ann Allergy Asthma Immunol 2016 May 26;116(5):392-3. Epub 2016 Mar 26.

Division of Allergy-Immunology, Northwestern University, Chicago, Illinois.

Ann Med 2016 26;48(4):256-67. Epub 2016 Mar 26.

c Department of Internal Medicine , Academic Medical Center, University of Amsterdam , The Netherlands.

Uncontrolled generation of bradykinin (BK) due to insufficient levels of protease inhibitors controlling contact phase (CP) activation, increased activity of CP proteins, and/or inadequate degradation of BK into inactive peptides increases vascular permeability via BK-receptor 2 (BKR2) and results in subcutaneous and submucosal edema formation. Hereditary and acquired angioedema due to C1-inhibitor deficiency (C1-INH-HAE and -AAE) are diseases characterized by serious and potentially fatal attacks of subcutaneous and submucosal edemas of upper airways, facial structures, abdomen, and extremities, due to inadequate control of BK generation. A decreased activity of C1-inhibitor is the hallmark of C1-INH-HAE (types 1 and 2) due to a mutation in the C1-inhibitor gene, whereas the deficiency in C1-inhibitor in C1-INH-AAE is the result of autoimmune phenomena. Read More

Ann Allergy Asthma Immunol 2016 May 24;116(5):472-3. Epub 2016 Mar 24.

Division of Hematopathology, Mayo Clinic, Rochester, Minnesota.

Clin Exp Immunol 2016 Jul 13;185(1):81-5. Epub 2016 Apr 13.

Department of Internal Medicine, Grenoble University Hospital.

Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. Read More

World Allergy Organ J 2016 8;9. Epub 2016 Feb 8.

Department of Dermatology, Venereology and Allergology, University Medical Center, Georg August University Göttingen, Göttingen, Germany ; Lower Saxony Institute of Occupational Dermatology, University Medical Center Göttingen and University of Osnabrück, Osnabrück, Germany ; Dermatology Office, Elmshorn, Germany.

Background: There exist several reports of atopy and allergen-specific IgE-mediated hypersensitivity transferred by bone marrow transplantation, and it has been concluded that the transfer of allergic reactivity results from adoptive transfer of IgE-producing donor-derived B- and/or plasma cells. To the best of our knowledge we report the first case of peanut allergy after PBSCT.

Case Presentation: A 55-year-old anciently non allergic man with secondary acute myeloid leukemia (AML) received an allogeneic peripheral blood stem cell transplantation from a matched unrelated donor following reduced-intensity conditioning. Read More

Mol Immunol 2016 Mar 18;71:161-5. Epub 2016 Feb 18.

Centre de Référence des Angioedèmes CREAK, CHU Grenoble, CHU Angers and AP-HP, Paris, France; Université Joseph Fourier, GREPI EA-UJF 7408, Grenoble, France.

C1 Inhibitor (C1Inh), a member of the Serine proteinase inhibitor family, is the most heavily glycosylated plasma protein. This work investigated the impact of C1Inh glycosylation on its function regarding protease targets and autoantibody binding. C1Inh deglycosylation was found to affect its function with O-linked polysaccharides, but not with N-linked polysaccharides, in controlling the contact phase but not C1s target, thus indicating the N-terminal domain's involvement in C1Inh function. Read More

Pol Arch Med Wewn 2016 ;126(1-2):76-85

Angioedema and urticaria often constitute a challenge in daily clinical practice. They may either co- -occur or present as independent conditions. They are characterized by a complex pathomechanism, and their symptoms may be triggered by diverse factors. Read More

BMJ Case Rep 2016 Jan 28;2016. Epub 2016 Jan 28.

Department of Immunology, The Canberra Hospital, Woden, Australian Capital Territory, Australia ANU Medical School, Australian National University, Acton, Australian Capital Territory, Australia.

A man in his 60s was brought to the emergency department, with airway compromise and dysarthria due to a grossly enlarged tongue. As he was on a current course of antibiotics, he was treated for a likely antibiotic-associated allergic reaction. However, as he failed to improve with intramuscular and nebulised epinephrine, another cause of his symptoms was sought. Read More

Transfusion 2016 May 12;56(5):1022-9. Epub 2016 Jan 12.

Department of Oto-Rhino-Laryngology, Technische Universität München, München, Germany.

Background: Patients suffering from bradykinin-induced angioedema show recurrent swelling of subcutaneous and submucosal structures. Increased bradykinin levels lead to an increase in vascular permeability and edema formation. Current therapy consists of B2 bradykinin receptor antagonists, C1-esterase-inhibitor (C1-INH) concentrate, or the kallikrein inhibitor ecallantide. Read More

Br J Haematol 2016 Mar 5;172(6):902-8. Epub 2016 Jan 5.

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

Marginal zone lymphoma represents about 10% of all non-Hodgkin lymphomas (NHLs). 33% of patients with acquired angioedema (AAE) due to acquired C1-inhibitor (C1-INH) deficiency (C1-INH-AAE) have or will develop NHLs. C1-INH-AAE is a rare condition. Read More

Iran J Allergy Asthma Immunol 2015 Dec;14(6):642-5

Medical University of Pleven, Faculty of Medicine Pleven, Bulgaria.

Angioedema is an asymmetric non-pitting oedema on face, lips, tongue and mucous membranes; any delay in diagnosis and treatment can be fatal. Treatment with lisinopril as an angiotensin converting enzyme (ACE) inhibitor, can be a reason of angioedema. Here we report a case who developed oral-facial edema four years after using lisinopril/hydrochlorothiazide. Read More

HNO 2015 Dec;63(12):885-93; quiz 894-5

Universitätsklinik für Hals-Nasen-Ohrenheilkunde, Kopf- und Halschirurgie, Universitätsklinikum Ulm, Frauensteige 12, 89070, Ulm, Deutschland.

The incidence of bradykinin-induced angioedema is considerably lower than that of histamine-induced forms; however, the same is true for the clinician's knowledge of this condition. Bradykinin-induced angioedemas include hereditary angioedema (HAE), as well as acquired forms induced by drugs or antibody formation, e.g. Read More

Medicine (Baltimore) 2015 Nov;94(45):e1939

Service des Urgences, Centre de Référence associé sur les angiœdèmes à kinines (CRéAk), Assistance Publique-Hôpitaux de Paris, Hôpital Louis Mourier, Université Paris 7, 92 700 Colombes (NJ, JA); Urgences et (P-GR, FL, LH, FA); SAMU-SMUR 93, Assistance Publique - Hôpitaux de Paris, Hôpital Avicenne, Université Paris 13, 93 000 Bobigny (P-GR, FL, FA); Urgences, Assistance Publique - Hôpitaux de Paris, Hôpital Jean Verdier, Université Paris 13, 93 140 Bondy (AL, AK); Urgences, Hôpital d'Eaubonne, 95 600 Eaubonne (AL); Urgences, Hôpital de Gonesse, 95 500 Gonesse (MY); Service de Médecine Interne, DHUi2B, Centre de Référence associé sur les angiœdèmes à kinines (CRéAk), Assistance Publique - Hôpitaux de Paris, Hôpital Saint-Antoine, Université Paris 6, 75 012 Paris, France (OF).

The number of cases of acquired angioedema related to angiotensin converting enzyme inhibitors induced (ACEI-AAE) is on the increase, with a potential concomitant increase in life-threatening attacks of laryngeal edema. Our objective was to determine the main characteristics of ACEI-AAE attacks and, in doing so, the factors associated with likelihood of hospital admission from the emergency department (ED) after a visit for an attack.A prospective, multicenter, observational study (April 2012-December 2014) was conducted in EDs of 4 French hospitals in collaboration with emergency services (SAMU 93) and a reference center for bradykinin-mediated angioedema. Read More

Allergy Asthma Proc 2015 Nov-Dec;36(6):468-72

Department of Pediatric Allergy and Asthma, Gazi University School of Medicine, Ankara, Turkey.

Background: The association between thyroid autoimmunity (TA) and idiopathic isolated angioedema (or angioedema without urticaria) has not been evaluated in either children or in adults up until now. We, therefore, aimed to investigate underlying or concomitant TA and/or autoimmune thyroid disease in children diagnosed with recurrent idiopathic angioedema.

Methods: Children who were consecutively diagnosed with recurrent idiopathic histaminergic acquired angioedema (IH-AAE) between January 2011 and January 2014 constituted the case group. Read More

Neth J Med 2015 Oct;73(8):383-5

Department of Hematology, Radboud University Hospital, Nijmegen, the Netherlands.

Introduction: Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor.

Case Report: A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody. Read More

Clin Immunol 2015 Dec 1;161(2):225-40. Epub 2015 Sep 1.

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA. Electronic address:

Around 350 million people worldwide suffer from rare diseases. These may have a genetic, infectious, or autoimmune basis, and several include an inflammatory component. Launching of effective treatments can be very challenging when there is a low disease prevalence and limited scientific insights into the disease mechanisms. Read More

Hautarzt 2015 Oct;66(10):723-5

Hautklinik des Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.

Acquired angioedema due to C1 inhibitor deficiency (C1-INH-AAE) is characterized by recurrent edema of the subcutaneous and/or submucosal tissue without wheals and negative family history of angioedema. Here, we present the case of a patient with a chronic lymphatic B cell leukemia who suffered from both C1-INH-AAE and chronic spontaneous urticaria. Oral corticosteroids, antihistamines, and the anti-IgE antibody omalizumab were applied to treat the chronic urticaria in combination with the plasma-derived C1 esterase inhibitor concentrate Berinert® and the bradykinin B2 receptor antagonist icatibant, but the symptoms did not improved significantly. Read More

J Immunol Methods 2015 Nov 28;426:114-9. Epub 2015 Aug 28.

Sanquin Diagnostic Services, Amsterdam, The Netherlands. Electronic address:

Background: Neutralizing autoantibodies (NAbs) against plasma serpin C1-inhibitor (C1-inh) are implicated in the rare disorder, acquired angioedema (AAE). There is insufficient understanding of the process of antibody formation and its correlation with disease progression and severity. We have developed an ELISA for detecting neutralizing capacity of anti-C1-inh positive plasma samples that can be used to study changes in NAb repertoire in patient plasma over the course of disease. Read More

Iran J Med Sci 2015 Jul;40(4):372-5

Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Read More

Clin Chem Lab Med 2016 Feb;54(2):207-14

Several clinical and biological features of lymphoproliferative diseases have been associated with an increased risk of developing autoimmune manifestations. Acquired deficiency of C1-inhibitor (C1-INH) (AAE) is a rare syndrome clinically similar to hereditary angioedema (HAE) characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. Bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein (a serine protease controlled by C1-INH), is the mediator of symptoms. Read More