Search Our Scientific Publications & Authors

Medicina (Kaunas) 2022 Jun 17;58(6). Epub 2022 Jun 17.

Dermatology Department, "Elias" University Emergency Hospital, 011461 Bucharest, Romania.

Chronic spontaneous urticaria (CSU) considerably alters patients' quality of life, often for extended periods, due to pruriginous skin lesions, impaired sleep, unexpected development of angioedema, and failure of conventional treatments in properly controlling signs and symptoms. Recent research focused on the development of new therapeutic agents with higher efficacy. Although the production of specific immunoglobulin E (IgE) antibodies against certain allergens is not a characteristic of the disease, treatment with omalizumab, a monoclonal anti-IgE antibody, proved efficient and safe in patients with moderate to severe chronic spontaneous urticaria uncontrolled by H1-antihistamines. Read More

J Allergy Clin Immunol Pract 2022 May 6. Epub 2022 May 6.

Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany. Electronic address:

Background: Aquagenic urticaria (AquaU) is a rare variant of chronic inducible urticaria where wheals occur after skin contact with water. Information on clinical manifestations and treatment outcomes is limited, which makes the management of AquaU challenging.

Objective: To systematically review disease features and relevant triggers of AquaU and patients' response to treatment. Read More

Front Immunol 2022 19;13:843718. Epub 2022 Apr 19.

Clinical Immunology, Angioedema and Allergy Unit, The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Ramat Gan, Israel.

Background: Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on re-thrombosis following APS-diagnosis are limited.

Methods: This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel. Read More

Eur J Dermatol 2022 02;32(1):49-55

CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France, Centre de Référence des Angiœdèmes à Kinines, F-59000 Lille, France, University Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000 Lille, France, Inserm, F-59000 Lille, France.

JAAPA 2022 May;35(5):36-38

Arielle D. Zwanziger practices in family medicine at Kaiser Permanente of Washington in Seattle. The author has disclosed no potential conflicts of interest, financial or otherwise.

Abstract: Acquired angioedema is a rare disorder characterized by nonurticarial angioedema secondary to deficiency or altered activity of C1-esterase inhibitor protein. This article describes a patient whose recurrent angioedema was initially diagnosed as angiotensin-converting enzyme (ACE) inhibitor-induced angioedema. However, after further testing, she was diagnosed with acquired angioedema and subsequently treated with a synthetic bradykinin B2-receptor antagonist. Read More

Perm J 2021 11 22;25. Epub 2021 Nov 22.

Allergy Department, Southern California Permanente Medical Group, Kaiser Permanente Southern California, San Diego, CA.

A new episode of urticaria and/or angioedema can be an anxiety-inducing event for both the patient and the physician(s) seeing them in primary care, urgent care, or the emergency department. These events are commonly mistaken for "allergic" reactions and often mistreated. The most common causes of new onset urticaria in older children and adults, with or without angioedema, and normal vital signs or hypertension, are post-infection or acute idiopathic urticaria. Read More

Int Arch Allergy Immunol 2022 24;183(5):572-577. Epub 2022 Mar 24.

Clinical Immunology, Faculdade de Medicina, Centro Universitário FMABC, Santo Andre, Brazil.

Background: Acquired deficiency of C1 inhibitor (AAE-C1-INH) is a very rare cause of recurrent angioedema, with few cases reported in the literature. We aimed to describe a series of patients with AAE-C1-INH who were diagnosed and received care at angioedema reference centers in Brazil, affiliated to the Brazilian Group of Studies on Hereditary Angioedema.

Methods: Fourteen patients from 8 Brazilian Angioedema Reference Centers, diagnosed with AAE-C1-INH, were included in this study. Read More

Int J Rheum Dis 2022 May 3;25(5):617-625. Epub 2022 Mar 3.

Kocaeli University Medical Faculty, Division of Child Neurology, Department of Pediatrics, Izmit, Turkey.

Background: Juvenile dermatomyositis (JDM) is the most common subtype of idiopathic inflammatory myopathies in childhood. Gottron's papules, shawl sign, periorbital heliotrope rash, and periungual telengiectasis are characteristic skin findings of the disease. Besides characteristic skin involvement, some other skin findings, such as angioedema, may be seen prior or in the course of the disease. Read More

J Investig Allergol Clin Immunol 2022 Feb 11. Epub 2022 Feb 11.

Dept. of Medicine, Division of Pulmonary, Critical Care, Allergy and Immunology, Medical University of South Carolina, Charleston, SC USA.

Case Rep Oncol 2021 Sep-Dec;14(3):1806-1813. Epub 2021 Dec 23.

Medical Oncology Department, Liverpool Hospital, Liverpool, New South Wales, Australia.

Small cell carcinoma is associated with a number of paraneoplastic syndromes. We report a case of a 42-year-old female who presented with primary laryngeal small cell carcinoma associated with concurrent paraneoplastic dermatomyositis and paraneoplastic angioedema secondary to acquired C1 esterase inhibitor deficiency. The patient required extensive treatment for her dermatomyositis including high-dose corticosteroid therapy and intravenous immunoglobulin followed by steroid-sparing disease-modifying immunosuppression. Read More

Allergy Asthma Clin Immunol 2022 Jan 13;18(1). Epub 2022 Jan 13.

University of Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, French National Center of Reference for Angioedema, 59000, Lille, France.

Background: Hereditary angioedema (HAE) is characterized by potentially severe and life-threatening attacks of localized swelling. Prophylactic therapies are available, including attenuated androgens. Efficacy of attenuated androgens has not been assessed in large, randomized, placebo-controlled trials and can be associated with frequent, and sometimes severe, side effects. Read More

Cureus 2021 Nov 30;13(11):e20041. Epub 2021 Nov 30.

Internal Medicine Department, Primary Health Care Corporation, Athens, GRC.

Dulaglutide is an injectable glucagon-like peptide-1 receptor agonist approved for the treatment of adults with type 2 diabetes. Angioedema is defined as self-limiting edema, localized in the deeper layers of the skin and mucosa. Angioedema can be hereditary or acquired which can be allergic due to reactions to foods, insect bites and stings, and latex, drug-induced, caused by physical stimuli and associated with lupus erythematosus and hypereosinophilia. Read More

Clin Transl Allergy 2021 Dec;11(10):e12083

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

Background: Angioedemas localized in the upper airway are potentially life threatening, and without proper treatment, they may lead to death by suffocation. Upper airway edemas (UAE) in bradykinin-mediated angioedemas can even be the first symptoms of the disease.

Methods: Our survey was performed with a retrospective long-term follow-up method from the medical history of 197 hereditary (C1-INH-HAE) and 20 acquired C1-inhibitor deficiency (C1-INH-AAE), 3 factor XII and 3 plasminogen gene mutation (FXII-HAE, PLG-HAE) patients treated at our center between 1990 and 2020. Read More

Front Immunol 2021 8;12:785736. Epub 2021 Dec 8.

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

Angioedema is a prevailing symptom in different diseases, frequently occurring in the presence of urticaria. Recurrent angioedema without urticaria (AE) can be hereditary (HAE) and acquired (AAE), and several subtypes can be distinguished, although clinical presentation is quite similar in some of them. They present with subcutaneous and mucosal swellings, affecting extremities, face, genitals, bowels, and upper airways. Read More

Indian Dermatol Online J 2021 Nov-Dec;12(6):796-804. Epub 2021 Nov 22.

Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Hereditary angioedema (HAE) is an uncommon disorder with a global prevalence of approximately 1 in 10,000 to 1 in 50,000 population. This disease is grossly underrecognized in India because of lack of awareness and/or lack of diagnostic facilities. Clinical manifestations include swelling over face, eyes, lips, hands, feet, and genitals, abdominal pain, and life-threatening laryngeal edema. Read More

Allergy Asthma Clin Immunol 2021 Dec 19;17(1):134. Epub 2021 Dec 19.

Division of Allergy and Immunology, Department of Medicine, University of British Columbia, 905-750 Broadway W, Vancouver, BC, V5Z 1H8, Canada.

Background: Hereditary angioedema (HAE) is an inherited condition manifesting as recurrent angioedema episodes which is caused by deficiency or dysfunction of C1 inhibitor. Although complement dysregulation has historically been shown to be associated with various malignancy and immune disorders, it is currently not known if HAE patients are at an increased risk of developing malignancy or autoimmune conditions.

Case Presentation: We reviewed the charts of 49 HAE patients and identified 6 patients who had a co-existing malignancy diagnosis (two with breast cancer, one with melanoma, one with pancreatic cancer, one with renal cancer and one with cervical dysplasia) and 6 patients who had a diagnosis of a co-existing immune disorder (two with rheumatoid arthritis, two with ulcerative colitis, one with chronic urticaria with hypothyroidism and one with Sjogren's syndrome). Read More

Braz J Anesthesiol 2021 Nov 27. Epub 2021 Nov 27.

Centro Hospitalar Vila Nova de Gaia/Espinho, Department of Anaesthesiology, Vila Nova de Gaia, Portugal.

Acquired angioedema with C1 inhibitor deficiency (AAE-C1INH) is a very rare condition of bradykinin-mediated angioedema. One of its major complications is potentially life-threatening, laryngeal edema. We report a 53-year-old woman with AAE-C1INH proposed for an elective broncofibroscopy. Read More

J Allergy Clin Immunol Pract 2022 04 26;10(4):1020-1028. Epub 2021 Nov 26.

Hospital General Universitario Gregorio Marañón, Allergy Section, Madrid, Spain; Institute for Health Research Gregorio Marañón, Madrid, Spain.

Background: Data on acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) from 4 European countries (France, Italy, Germany, and Hungary) were recently published.

Objective: To report data from a group of 50 patients with acquired C1-INH deficiency from Spain, of whom 46 had angioedema, and compare them with other European series.

Methods: We performed a retrospective observational study of 46 patients with C1-INH-AAE and 4 asymptomatic patients. Read More

Pharmaceuticals (Basel) 2021 Nov 19;14(11). Epub 2021 Nov 19.

JSC "GENERIUM", 14, Vladimirskaya Street, Volginskiy 601125, Vladimir Region, Russia.

The disfunction or deficiency of the C1 esterase inhibitor (C1INH) is associated with hereditary or acquired angioedema (HAE/AAE), a rare life-threatening condition characterized by swelling in the skin, respiratory and gastrointestinal tracts. The current treatment options may carry the risks of either viral infection (plasma-derived Berinert) or immune reaction (human recombinant C1INH from rabbit milk, Ruconest). This study describes the physicochemical and biological characterization of a novel recombinant human C1 esterase inhibitor (rhC1INH) from Chinese hamster ovary (CHO) cells for the treatment of hereditary angioedema compared to the marketed products Berinert and Ruconest. Read More

J Crit Care Med (Targu Mures) 2021 Apr 12;7(2):136-140. Epub 2021 May 12.

George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Mures Romania.

Introduction: Laryngeal oedema caused by acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a life-threatening condition. The swelling is bradykinin mediated and will not respond to the usual treatment with antihistamines, corticosteroids, or epinephrine. Instead, kallikrein-bradykinin-targeted therapies should be used promptly to prevent asphyxiation. Read More

Rev Alerg Mex 2021 Jul-Sep;68(3):206-208

Irmandade da Santa Casa de Misericórdia de São Paulo, Sector de Alergias e Inmunodeficiencias, São Paulo, Brasil.

Introduction: Diagnosis and treatment of hereditary angioedema (HAE) are necessary to improve the quality of life and even the survival of patients.

Case Report: A 52-year-old woman with angioedema for 30 years, which affects the face, tongue, and hands. It is asymmetric, with neither pruritus nor urticaria, without response to antihistamines or corticosteroids, with spontaneous resolution in 48 hours to 72 hours; with a family history of angioedema. Read More

Clin Exp Immunol 2021 12 11;206(3):378-383. Epub 2021 Oct 11.

Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina, USA.

Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is rare and is associated with underlying lymphoproliferative diseases. C1-INH deficiency may be due to neoplastic over-consumption of C1-INH and the generation of anti-C1-INH autoantibodies. Uncovering an occult malignancy can lead to earlier oncology referral and improvement of angioedema after treatment of the underlying lymphoproliferative disorder. Read More

Indian J Pharmacol 2021 Jul-Aug;53(4):298-300

Department of Dermatology, IPGME and R and SSKM Hospital, Kolkata, West Bengal, India.

Moxifloxacin is a fluoroquinolone with excellent activity in community-acquired respiratory tract infections. Common adverse effects are gastrointestinal symptoms, headache, dizziness, etc., Some serious adverse effects include tendon rupture, rhabdomyolysis, peripheral neuropathy, and interstitial nephritis. Read More

Clin Immunol 2021 09 4;230:108819. Epub 2021 Aug 4.

Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL, USA. Electronic address:

Acquired angioedema due to C1 inhibitor deficiency (C1-INH-AAE) is a rare disease characterized by adult-onset recurrent non-urticarial angioedema with low levels of C1-INH. It is associated with lymphoproliferative disorders, and treatments are off-label with variable success. We conducted a systematic literature review to include patients with C1-INH-AAE identified in PubMed and Embase databases between January 2006 and February 2021. Read More

Intern Med J 2021 Aug 4. Epub 2021 Aug 4.

Department of Clinical Immunology and Allergy, Auckland City Hospital, Grafton Road Auckland, 1032.

Background: HAE leads to significant morbidity and mortality from unpredictable intermittent peripheral, abdominal and laryngeal swelling. Access to appropriate healthcare and effective therapies which can prevent and treat attacks, reduce the suffering and greatly improve quality of life. Although treatments such as C1 inhibitor (Berinert®), and Icatibant® are available in New Zealand (Aotearoa), there is no published data available on their use. Read More

Proc (Bayl Univ Med Cent) 2021 Feb 22;34(4):519-520. Epub 2021 Feb 22.

Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas.

A 75-year-old woman presented with recurrent abdominal pain and vomiting for 1 year and was later found to have splenomegaly and pancytopenia. This case report depicts a clinical picture of intestinal angioedema, a challenging diagnosis, and an underlying rare syndrome of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma. Read More

Allergy Asthma Proc 2021 07;42(4):317-324

From the Department of Otorhinolaryngology, Technische Universität München, München, Germany; and.

Hereditary angioedema (HAE) is a disease that leads to recurrent swelling of the skin and mucous membranes, including the upper airway tract. Apart from being deadly, these attacks can be debilitating, which leads to a poor quality of life in patients. Clinicians are occasionally confronted with patients who have recurrent attacks despite treatment with C1 esterase inhibitor concentrate or β₂-receptor antagonists. Read More

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 (Engl Ed) 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 10 20;9(10):3744-3751. 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