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ACE Inhibitor-Induced Angioedema

Renin-Angiotensin-Aldosterone System-Blocker-Induced Quincke Edema


  • Presenting complaints are often vague. Those most commonly described include swelling in the subcutaneous and submucosal tissues.[ncbi.nlm.nih.gov]
  • ., 2014 2014 Clinical expression of inflammatory bowel disease in Zadar county I Klarin Medicinski fakultet, Sveučilište u osijeku, 2014 2014 PP148-MON FIGHT AGAINST MALNUTRITION IN CROATIA: AN EXPERIENCE FROM NINE GASTROENTEROLOGICAL DEPARTMENTS Ž Krznarić[scholar.google.com.pk]
  • Ultimately, this results in the C1 inhibitor level falling, which leads to activation of the contact system, generation of bradykinin and angioedema. Dr. C : ACID is sometimes referred to as the “canary in the coal mine”. Could you explain this? Dr.[angioedemacenter.com]
Pleural Effusion
  • Abdominal computed tomography – This may show severe edema of the bowel wall [1] Chest radiography – This may show pleural effusion Soft-tissue neck radiography – This may show soft-tissue swelling [5] See Workup for more detail.[emedicine.medscape.com]
Abdominal Pain
  • INTRODUCTION: Acquired angioedema (AAE), an acquired deficiency of C1esterase inhibitor, is a medically treatable condition which can cause severe abdominal pain mimicking an acute surgical abdomen.[ncbi.nlm.nih.gov]
  • Sometimes there is abdominal pain due to edema of the gastrointestinal mucosa, and life-threatening edema of the upper respiratory tract.[checkorphan.org]
  • Interestingly, the severity of abdominal pain decreased and she no longer had associated symptoms such as nausea, vomiting and diarrhea.[bioline.org.br]
  • People with AAE can have edema of the lining of the digestive tract, which can cause abdominal pain and nausea, as well as edema of the upper airway, which can be life-threatening. [1] Swelling episodes may have various triggers, such as mild trauma ([rarediseases.info.nih.gov]
  • […] tissues occur throughout the body without the presence of urticaria Typically involves arms, legs, hands, trunk, face, mouth, larynx, airway, genitals, and tongue Gastrointestinal tract often involved, with recurrent episodes of cramping, abdominal pain, nausea[arupconsult.com]
  • Interestingly, the severity of abdominal pain decreased and she no longer had associated symptoms such as nausea, vomiting and diarrhea.[bioline.org.br]
  • Severe swelling in the intestines or digestive system can cause nausea, vomiting, and intense pain in the abdomen. When severe swellings occur in the mouth or throat, they can block the airways and make it difficult to breathe.[angioedemanews.com]
  • In hereditary angioedema (HAE) swellings frequently occur in the gastrointestinal tract (especially in the small and large bowel), which can result in severe abdominal pains, nausea, vomiting and diarrhoea even lasting several days.[aha.ch]
  • Ominous signs suggesting airway involvement include dysphagia, voice change, and stridor. There will generally be a family history of angio-oedema. However, 5% of adults carry the mutation for the C1-INH gene while remaining asymptomatic.[academic.oup.com]
Joint Swelling
  • On detailed history, patient had experienced loss of appetite and loss of weight (8 kg) over the past 3 months but denied fever, night sweats, chronic cough, joint swelling, oral ulcers and rashes.[ncbi.nlm.nih.gov]
  • We have recently studied a patient with rectal carcinoma who manifests a complement pattern compatible with the acquired form of angioedema. No previous personal or family history of allergic disease or angioedema was present.[ncbi.nlm.nih.gov]
  • Bullous Pemphigoid Bullous pemphigoid (BP) is a generalized blistering disorder characterized by pruritus and rigid, subepidermal blisters (Figure 2 D).[frontiersin.org]
  • Detection of active kallikrein in induced blister fluids of hereditary angioedema patients. J Exp Med. 1980;152(3):742–747. 14. Bowen T, Cicardi M, Bork K, et al.[dovepress.com]
Skin Lesion
  • Hereditary Angioedema Wheal response Urticaria Skin lesion List of cutaneous conditions[en.wikipedia.org]
  • Dermatitis Herpetiformis Dermatitis herpetiformis (DH), a celiac disease-related skin disorder, is characterized with itchy blistering skin lesions especially at elbows and knees.[frontiersin.org]


  • A literature review was completed for the purpose of assessing the diagnostic value of C1q when used in the workup of AAE.[ncbi.nlm.nih.gov]
  • Workup reveal low complements 3 and 4 (C3, C4), low normal C1q level, elevated ESR, and cryoglobulin which favor the diagnosis of acquired angioedema (AAE).[shmabstracts.com]
  • The differential diagnosis for angioedema without urticaria (“hives”) is fairly narrow, and requires a unique workup and management strategy.[medicalmediareview.com]
  • Although no association between the CLL and angioedema was initially recognized, further workup showed her to have low C1-INH levels. Chemotherapy helped prevent subsequent episodes, but three years later she redeveloped angioedema.[ecancer.org]
Pulmonary Infiltrate
  • Both the patient's peripheral blood mononuclear cells and a suspension of cells from the pulmonary infiltrate were capable of depleting the first component of complement and its inhibitor from homologous plasma.[ncbi.nlm.nih.gov]
Pleural Effusion
  • Abdominal computed tomography – This may show severe edema of the bowel wall [1] Chest radiography – This may show pleural effusion Soft-tissue neck radiography – This may show soft-tissue swelling [5] See Workup for more detail.[emedicine.medscape.com]


  • C1-INH concentrates and attenuated androgens are used for short-term prophylactic treatment. Long-term prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics.[ncbi.nlm.nih.gov]


  • Prognosis The prognosis depends on the risk of developing hematological manifestations. Rituximab can be an alternative treatment in cases of AAE associated with C1-INH antibodies.[orpha.net]
  • Prognosis - Acquired angioedema Treatment - Acquired angioedema Different drugs are available for the treatment of acquired angioedema.[checkorphan.org]
  • The prognosis of all types of angioedema is generally good with treatment. However, with acquired angioedema, an underlying immune system problem is usually present, causing the swellings and affecting the individual’s overall health.[angioedemanews.com]
  • Prognosis The prognosis for patients with angioedema depends on the etiology and varies as follows: Angioedema with identifiable causes – If the trigger(s) can be identified and avoided, angioedema can be prevented Angioedema without identifiable causes[emedicine.medscape.com]


  • Histamine is the mediator in acquired angioedema of unknown etiology (idiopathic histaminergic acquired angioedema ), whereas in other forms the main mediator is bradykinin.[pesquisa.bvsalud.org]
  • Etiology The edemas are triggered by increased permeability of the blood vessels in response to elevated levels of bradykinin as a result of the C1-INH deficiency.[orpha.net]
  • Etiology Angiotensin-Converting Enzyme inhibitors (Acute ACE inhibitor-induced angioedema) : think about this diagnosis in any patient on ACE inhibitors for any length of time who presents with otherwise-unexplained recurrent abdominal pain.[medicalmediareview.com]
  • Hence, etiology of AAE was determined to be IgM-monoclonal gammopathy of undetermined significance (MGUS).[hoajonline.com]


  • Summary Epidemiology Prevalence is unknown. Clinical description Onset most commonly occurs after 50 years of age.[orpha.net]
  • […] lymphomas) Autoimmune disease Neoplasia Urticarial vasculitis associated with angioedema Idiopathic or cold-induced angioedema Cellulitis Parasitic infection (eg, Trichinella spp) Autoimmune conditions Laryngeal edema – peritonsillar abscess Background Epidemiology[arupconsult.com]
  • Collegium antropologicum 35 (3), 781-785, 2011 11 2011 Epidemiology and clinical presentation of inflammatory bowel disease in Zadar County, Croatia I Klarin, L Majnarić, Ž Jovanović, D Nakić, I Včev, A Včev Collegium antropologicum 37 (4), 1161-1170,[scholar.google.hr]
  • Epidemiology Angio-oedema is common, but varies with the population investigated. Lifetime prevalence rates of 8.8% have been reported [ 6 ]. The most common variety is recurrent idiopathic angio-oedema.[patient.info]
Sex distribution
Age distribution


  • This review summarizes the clinical aspects, pathophysiology, and management of AAE compared with the types of hereditary angioedema.[ncbi.nlm.nih.gov]
  • Future research needs to address its underlying pathophysiologic mechanisms and its clinical significance in treatment and course of pSLE.[acrabstracts.org]
  • Supporting the importance of bradykinin in acquired angioedema, vascular permeability has been shown to increase in mice deficient in C1-INH, but not in mice with a deficiency in both C1-INH and the bradykinin B2 receptor. [3] The precise pathophysiology[emedicine.medscape.com]
  • The pathophysiology of hereditary angioedema. Clin Immunol. 2004;114:3-9. Bower T, et al., Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema. J Allergy Clin Immunol. 2004;114:629-37.[rarediseases.org]
  • 1 – low complement 1 esterase inhibitor (C1-INH) level and low function (85%) If C1q is also low, suspect acquired angioedema Type 2 – normal C1-INH level but low function (15%) Type 3 (familial angioedema) – normal C1-INH and normal function (rare) Pathophysiology[arupconsult.com]


  • CONCLUSION: Wider appreciation of the possibility of AAE, particularly in patients with lymphoproliferative disorders, could lead to preventive therapy and spare unnecessary surgery.[ncbi.nlm.nih.gov]
  • Knowledge of this rare disease and anticipation of the link between CLL and AAE can prevent further attacks and associated morbidity. Loading Article Metrics ... Please wait[ecancer.org]

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