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    476 results match your criteria Angioedema Acquired

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    [Angioedema and the role of bradykinins: new treatments and implications in patients with heart failure].
    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

    Recurrent angioedema associated with pharmacological inhibition of dipeptidyl peptidase IV.
    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

    Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.
    Clin Exp Immunol 2016 Dec 9. Epub 2016 Dec 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

    Differential Diagnosis of Chronic Urticaria and Angioedema Based on Molecular Biology, Pharmacology, and Proteomics.
    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

    Idiopathic Non-histaminergic Angioedema: Successful Treatment with Omalizumab in Five Patients.
    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

    [Angioedema].
    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

    Overview of Laboratory Testing and Clinical Presentations of Complement Deficiencies and Dysregulation.
    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

    Perioperative course in patients with hereditary or acquired angioedema.
    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

    A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients.
    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

    Monoclonal gammopathy of cutaneous significance: review of a relevant concept.
    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

    Novelties in the Diagnosis and Treatment of Angioedema.
    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

    Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema.
    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

    Angioedema: Clinical Presentations and Pharmacological Management.
    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

    Clinical presentation, pathophysiology, diagnosis, and treatment of acquired and hereditary angioedema: Exploring state-of-the-art therapies in RI.
    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

    "Nuts and Bolts" of Laboratory Evaluation of Angioedema.
    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

    The Use of Plasma-Derived Complement C1-Esterase Inhibitor Concentrate (Berinert®) in the Treatment of Angiotensin Converting Enzyme-Inhibitor Related Angioedema.
    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

    Angioedema Phenotypes: Disease Expression and Classification.
    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

    Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment.
    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

    Idiopathic histaminergic angioedema without wheals: a case series of 31 patients.
    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

    A lurking threat: transfer of peanut allergy through peripheral blood stem cell transplantation.
    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

    C1 Inhibitor as a glycoprotein: The influence of polysaccharides on its function and autoantibody target.
    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

    'Epinephrine-resistant' angioedema.
    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

    Nanofiltrated C1-esterase-inhibitor in the prophylactic treatment of bradykinin-mediated angioedema.
    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

    High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibtor deficiency.
    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

    Angiotensin Converting Enzyme Inhibitor-related Angioedema: A Case of an Unexpected Death.
    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

    [Bradykinin-induced angioedema: Definition, pathogenesis, clinical presentation, diagnosis and therapy].
    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

    Angioedema Related to Angiotensin-Converting Enzyme Inhibitors: Attack Severity, Treatment, and Hospital Admission in a Prospective Multicenter Study.
    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

    Association between thyroid autoimmunity and recurrent angioedema in children.
    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

    Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?
    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

    Applying complement therapeutics to rare diseases.
    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

    [Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia].
    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

    ELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients.
    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

    Refractory Angioedema in a Patient with Systemic Lupus Erythematosus.
    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

    The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity.
    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

    [Hereditary angiœdema treatments: Recommendations from the French national center for angiœdema (Bordeaux consensus 2014)].
    Presse Med 2015 May 29;44(5):526-32. Epub 2015 Apr 29.
    CHU de Grenoble, centre national de référence des angiœdèmes, 38043 Grenoble cedex 09, France; Université Paris 6, AP-HP, hôpital Saint-Antoine, service de médecine interne, 75012 Paris, France.
    Introduction: Treatment of hereditary angiœdema (HAE), associated or not with C1Inh deficiency, has dramatically changed these last five years. Four new therapeutic options are now available in France. The French Reference Centre for Angioedema has a consensus meeting to give recommendations about their use. Read More

    Management of acute attacks of hereditary angioedema: role of ecallantide.
    J Blood Med 2015 16;6:115-23. Epub 2015 Apr 16.
    Department of Pediatrics, University of Utah, Salt Lake City, UT, USA ; Division of Allergy, Immunology and Rheumatology, University of Utah, Salt Lake City, UT, USA.
    Hereditary angioedema (HAE) is characterized as an episodic swelling disorder with autosomal dominant inheritance. Clinical features include nonpitting edema of external or mucosal body surfaces, and patients often present with swelling of the extremities, abdominal pain, and swelling of the mouth and throat, which can lead to asphyxiation. Patients with HAE classically have no associated urticaria, which is often referred to as nonhistaminergic angioedema. Read More

    Towards a specific marker for acute bradykinin-mediated angioedema attacks: a literature review.
    Eur J Dermatol 2015 Jul-Aug;25(4):290-5
    Internal Medicine Department,, Centre national de référence des angiœdèmes (CREAK),, Universités des Alpes-Grenoble 1, 38041 Grenoble, France, CEA, DSV/iRTSV,, INSERM, Unité 1036, Biologie du cancer et de l'infection, 17, rue des Martyrs, 38054 Grenoble, France.
    Background: Bradykinin-mediated angioedema (AE) is a rare disease characterised by recurrent angioedema linked to acquired (e.g. angiotensin converting enzyme inhibitor induced AE) or hereditary disorders (e. Read More

    Massive macroglossia secondary to angioedema: a review and presentation of a case.
    J Oral Maxillofac Surg 2015 May 7;73(5):905-17. Epub 2015 Jan 7.
    Regents Professor, Department of Oral and Maxillofacial Surgery, Texas A&M University, Baylor College of Dentistry, and the Department of Surgery, Division of Oral and Maxillofacial Surgery, Baylor University Medical Center, Dallas, TX.
    Macroglossia is a rare condition, but can severely affect the oral and maxillofacial region. Angioedema is a condition resulting from multiple mechanisms, all of which can result in macroglossia. This report describes an unusual case of acquired macroglossia in an adult resulting from chronic edema secondary to angioedema in the setting of stroke. Read More

    Recurrent intestinal obstruction with acquired angio-oedema, due to C1-esterase inhibitor deficiency.
    J Assoc Physicians India 2014 Jun;62(6):524-5
    A 52-year male with past history of ulcerative colitis 20 years back (now in remission), developed recurrent small intestinal obstruction at intervals of a few months. CT scan did not detect the cause initially. A repeat CT scan (USA) showed interbowel fluid with transient ascites (serum albumin normal). Read More

    Facial angioedema and systemic lupus erythematosus: case report.
    J Oral Maxillofac Surg 2015 May 13;73(5):928-32. Epub 2014 Dec 13.
    Director, Salivary Gland Center; Associate Dean and Clinical Professor, Department of Oral and Maxillofacial Surgery, Columbia University College of Dental Medicine, New York-Presbyterian Medical Center (Columbia Campus), New York, NY. Electronic address:
    Non-medication-related acquired deficiencies of C1 esterase inhibitor (C1-INH) can cause the facial acquired angioedema (AAE) seen in systemic lupus erythematosus (SLE). The defect can originate from a lymphoproliferative disease (LPD) that catabolizes C1-INH or from circulating antibodies that inactivate C1-INH. This report describes a third and rare variety of facial AAE originating in SLE in which there was no LPD or circulating antibodies to impede C1-INH activity. Read More

    Characterization of patients with angioedema without wheals: the importance of F12 gene screening.
    Clin Immunol 2015 Apr 2;157(2):239-48. Epub 2015 Mar 2.
    Department of Medical Sciences "M. Aresu", University of Cagliari, Italy.
    Sporadic and familiar forms of non-histaminergic angioedema and normal C1 inhibitor encompass a group of disorders possibly caused by bradikinin. We aimed to study the subgroups of hereditary angioedema with FXII mutation (FXII-HAE), unknown genetic defect (U-HAE) and idiopathic non-histaminergic acquired angioedema (InH-AAE). We screened the F12 locus in our cohort and delineated the clinical, laboratory and genetic features. Read More

    Distinct conditions support a novel classification for bradykinin-mediated angio-oedema.
    Dermatology 2015 20;230(4):324-31. Epub 2015 Feb 20.
    Department of Dermatology, Angers Hospital, L'UNAM University, Angers, France.
    Background: Angio-oedema (AO) can be attributable to bradykinin (BK) accumulation, as is the case for prototypical hereditary AO (HAO) due to C1 inhibitor (C1-INH) deficiency. However, our clinical experience in a reference centre has shown that some patients display a clinical history suggestive of HAO, but exhibit normal C1-INH function, have no mutation in the causative genes associated with HAO (SERPING1, F12), and report no intake of drugs known to promote AO.

    Objective: We sought to determine the frequency and distribution of different AO subtypes suspected to be BK-mediated AO (BK-AO) and defined by clinical, history and biological criteria (enzyme activities implicated in BK formation and catabolism). Read More

    Bradykinin-mediated angioedema: factors associated with admission to an intensive care unit, a multicenter study.
    Eur J Emerg Med 2016 Jun;23(3):219-23
    aEmergency Department bInternal Medicine, Reference Center for Angioedema (CRéAk), Assistance Publique - Hôpitaux de Paris, Hôpital Jean Verdier, Groupe hospitalier Hôpitaux Universitaires Paris Seine-Saint-Denis, Université Paris Bondy cSAMU-SMUR 93 dClinical Research Department, Assistance Publique - Hôpitaux de Paris, Hôpital Avicenne, Groupe hospitalier Hôpitaux Universitaires Paris Seine-Saint-Denis, Université Paris Bobigny eDepartment of Anesthesiology and Intensive care Unit fInternal Medicine, Reference Center for Angioedema (CRéAk), Hôpital Edouard-Herriot, Hospices civils de Lyon gInternal Medicine, Reference Center for Angioedema (CRéAk), CHU de Grenoble, Grenoble hDepartment of Dermatology iIntensive Care Unit, Reference Center for Angioedema (CRéAk), UNAM Université, Hôpital d'Angers, Cedex jDepartment of Dermatology, Reference Center for Angioedema (CRéAk), CHU Gabriel-Montpied, Clermont-Ferrand kInternal Medicine, Centre Hospitalier du Centre de Bretagne, Pontivy lInternal Medicine, CHU de Caen, Caen, France.
    Objective: Bradykinin-mediated angioedema is characterized by transient attacks of localized edema of subcutaneous or submucosal tissues and can be life-threatening when involving the upper airways. The aim of this study was to determine the features of acute attacks that might be associated with admission to an ICU.

    Patients And Methods: We carried out a retrospective, multicenter, observational study in consecutive patients attending one of six reference centers in France for acute bradykinin-mediated angioedema attacks. Read More

    Complement system in dermatological diseases - fire under the skin.
    Front Med (Lausanne) 2015 29;2. Epub 2015 Jan 29.
    Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki , Helsinki , Finland ; Huslab, Helsinki University Central Hospital , Helsinki , Finland ; Research Programs Unit, Immunobiology, University of Helsinki , Helsinki , Finland.
    The complement system plays a key role in several dermatological diseases. Overactivation, deficiency, or abnormality of the control proteins are often related to a skin disease. Autoimmune mechanisms with autoantibodies and a cytotoxic effect of the complement membrane attack complex on epidermal or vascular cells can cause direct tissue damage and inflammation, e. Read More

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