Presenting complaints are often vague. Those most commonly described include swelling in the subcutaneous and submucosal tissues. [ncbi.nlm.nih.gov]

Case Presentation: A 61 years old male patient not known to have any medical problem presented to the emergency department after he noticed swelling in his mouth which increased in size to involve both lips, he reported loose bowel movements in the last [shmabstracts.com]

• Mucosal Edema

  Acute small-bowel mucosal edema following enalapril use. JAMA 1988; 259: 3131. 7. Makani H, Messerli FH, Romero J, et al. Meta-analysis of randomized trials of angioedema as an adverse event of renin-angiotensin system inhibitors. [medicinabuenosaires.com]

  The contact system is activated, kallikrein is generated that cleaves high molecular weight kinninogen producing bradykinin (primary mediator in AAE) which increases vascular permeability causing mucosal edema [ 5 ]. [hoajonline.com]

• Congestive Heart Failure

  Improvement in exercise tolerance and symptoms of congestive heart failure during treatment with candesartan cilexetil. Symptom, tolerability, response to exercise trial of candesartan cilexetil in heart failure (STRETCH) investigators. [ge-bu.nl]

• Stridor

  Two things that one aught to be particularly concerned about are laryngeal edema (stridor), which can lead to acute respiratory failure, and bowel wall edema, which can lead to gastrointestinal colic and small bowel obstruction. [medicalmediareview.com]

  In serious cases, stridor of the airway occurs, with gasping or wheezy inspiratory breather sounds and decreasing oxygen levels. Intubation is required in these situations to forbid respiratory arrest and danger of death. [streetdirectory.com]

  Difficulty breathing or stridor indicates an emergency condition. Self-care includes cool compresses or soaks to the area to provide pain relief and reduce symptoms. [lymphedemapeople.com]

  Pruritus, urticaria, and bronchospasm do not occur, but laryngeal edema may be present, causing stridor (and sometimes death). Swelling resolves within about 1 to 3 days of onset. [merckmanuals.com]

  Examination Assess airway: Ensure patency and identify any obstructive symptoms (with, for example, stridor, dysphonia). Severe attacks can indicate the onset of systemic anaphylaxis and are characterised initially by dyspnoea or throat symptoms. [patient.info]

• Hoarseness

  However, when there is oedema around the head and neck, observation to detect airway involvement is required (for example, hoarseness). [patient.info]

  Visualization, ideally by nasopharyngeal laryngoscopy, should be considered in patients with stridor or hoarseness to evaluate the degree of laryngeal angioedema [ 21 ]. [intjem.springeropen.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]

  Interestingly, the severity of abdominal pain decreased and she no longer had associated symptoms such as nausea, vomiting and diarrhea. [bioline.org.br]

  Sometimes there is abdominal pain due to edema of the gastrointestinal mucosa, and life-threatening edema of the upper respiratory tract. [checkorphan.org]

• Nausea

  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]

• Dysphagia

  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]

• Heart Disease

  He suffered from ischemic heart disease and had transient cerebral ischemic attack. The episodes of angioedema were unrelated to his medications. [jrheum.org]

  Asphyxia due to angiotensin converting enzyme (ACE) inhibitor mediated angioedema of the tongue during the treatment of hypertensive heart disease. Journal of Forensic Sciences. 2001; 46:1239-43. 51. Pavletic A. [revista.asocolderma.org.co]

  Asphyxia due to angiotensin converting enzyme (ACE) inhibitor mediated angioedema of the tongue during treatment of hypertensive heart disease. J Forensic Sci 2001;46:1239–43. Schmidt TD, McGrath KM. [racgp.org.au]

• Heart Failure

  Effect of losartan compared with captopril on mortality in patients with symptomatic heart failure: randomised trial-the Losartan Heart Failure Survival Study ELITE II. Lancet 2000; 355; 1582-1587. [ge-bu.nl]

  For patients with less severe reactions to ACEi, ARBs may be used with close monitoring in patients with conditions that may particularly benefit from renin-angiotensin-aldosterone inhibition, such as chronic heart failure [ 36, 39 ]. [intjem.springeropen.com]

• Tongue Edema

  (Ishoo stage I). b Tongue edema (Ishoo stage III). [intjem.springeropen.com]

• Dermatitis

  Locations Boston: Allergy Associates Waltham: MGH Waltham Allergy Sokol, Caroline L., MD, PhD Clinical Interests Rhinosinusitis Urticaria Eczema (atopic dermatitis) Contact dermatitis (allergic) Asthma Food allergy and immune mediated disorders of the [massgeneral.org]

  الصفحة 516 - Werner Y, Lindberg M: Transepidermal water loss in dry and clinically normal skin in patients with atopic dermatitis. Acta Derm Venereol (Stockh) 1985;65:102-105. ‏ [books.google.com]

  […] disorder) Genetic testing – not necessary for diagnosis Differential Diagnosis Cutaneous edema Allergic urticaria/angioedema Drug-induced angioedema (eg, angiotensin-converting enzyme [ACE] inhibitors, nonsteroidal anti-inflammatory drugs [NSAIDs]) Contact dermatitis [arupconsult.com]

  Other skin conditions - eg, bullous pemphigoid, cellulitis and erysipelas, contact dermatitis, erythema multiforme, lymphoedema, morbilliform drug eruptions, SLE or urticaria pigmentosa. [patient.info]

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]

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]

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]

• Ventricular Hypertrophy

  Trans-thoracic echocardiogram demonstrated left ventricular hypertrophy, with heavy trabeculations, ejection fraction of 41%, and moderately enlarged left atrium. Cardiac MRI failed to meet criteria for non-compaction cardiomyopathy and amyloidosis. [alliedacademies.org]

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]

In angioedema, this examination is generally required to ensure adequate therapy, an opinion on prognosis and heredity, and preventive measures, and it should therefore be undertaken as a matter of course. DOI: 10.3238/arztebl.2017.0178a Prof. [aerzteblatt.de]

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]

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]

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]

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]

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]

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]

Treatment options include antihistamines and male steroids (androgens) that can also prevent the recurrent attacks. [m.kmle.co.kr]

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]

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]