O angioedema adquirido (AAE) é caracterizado pela ocorrência de edemas subcutâneos e/ou submucosos transitórios e recorrentes, resultando em inchaço e/ou dor abdominal devido à deficiência adquirida de um inibidor de C1 (C1-INH).
Introdução
O que você precisa saber de cara
O angioedema adquirido (AAE) é caracterizado pela ocorrência de edemas subcutâneos e/ou submucosos transitórios e recorrentes, resultando em inchaço e/ou dor abdominal devido à deficiência adquirida de um inibidor de C1 (C1-INH).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 9 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.
Membrane-bound metalloprotease which catalyzes the removal of a penultimate prolyl residue from the N-termini of peptides, such as Arg-Pro-Pro. May play a role in the metabolism of the vasodilator bradykinin
Cell membrane
Angioedema induced by ACE inhibitors
A potentially life-threatening side effect of ACE inhibitors that appears in a subset of patients taking these drugs for hypertension and cardiovascular disease treatment. AEACEI is characterized by swelling of the face, lips, tongue, and airway that can lead to suffocation and death if severe.
Variantes genéticas (ClinVar)
173 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
1 via biológica associada aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Angioedema adquirido
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
7 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Effect of Recombinant Tissue-Type Plasminogen Activator on Peripheral Blood Mononuclear Cells of Patients With Alteplase-Associated Angioedema.
Recombinant tissue-type plasminogen activator (R-tPA) is a thrombolytic agent used to treat acute ischemic stroke (IS). A rare but serious adverse effect of R-tPA is angioedema, which is characterized by plasma extravasation and increased release of vasoactive factors such as bradykinin, vascular endothelial growth factor A (VEGF-A), CXCL8, angiopoietin-1 (ANGPT-1), and ANGPT-2. Objective: To investigate whether R-tPA modulates immune cell activity differently in IS patients with and without angioedema, focusing on the release of vasoactive mediators from human peripheral blood mononuclear cells (PBMCs). PBMCs were isolated from 7 healthy controls (HCs), 7 IS patients without angioedema, and 7 IS patients who developed angioedema during R-tPA treatment (ISAE). The production and/or release of CXCL8, VEGF-A, ANGPT-1, and ANGPT-2 following ex vivo stimulation with R-tPA was measured. Plasma levels of these mediators were also assessed in ISAE patients during both angioedema attacks and remission. R-tPA inhibited the spontaneous release of VEGF-A, ANGPT-1, and ANGPT-2 from the PBMCs of HCs and IS patients. In contrast, a significant increase in the release of these mediators after stimulation with R-tPA was observed in PBMCs from ISAE patients. Plasma concentrations of all 4 mediators were higher during angioedema attacks than in remission, with a statistically significant elevation recorded for ANGPT-2. These preliminary data suggest that R-tPA-related angioedema may result from abnormal immune cell activation, leading to increased release of vasoactive mediators. This immune dysregulation may contribute to the pathophysiology of angioedema in susceptible IS patients treated with R-tPA.
Alteplase and Angioedema: Can Clinical Exome Sequencing Redefine the Paradigm?
Intravenous thrombolysis with recombinant tissue-type plasminogen activator (tPA) remains a keystone of acute ischemic stroke treatment but in a subset of patients is complicated by angioedema, a potentially life-threatening adverse event largely mediated by bradykinin signaling. The unpredictable and idiosyncratic nature of this reaction has long suggested an underlying genetic contribution, yet its molecular architecture has remained poorly characterized. We hypothesized that alteplase-associated angioedema represents a multigenic susceptibility phenotype, arising from the convergence of rare genetic variants across multiple interacting physiological systems rather than from a single causal variant. To explore this hypothesis, we performed clinical exome sequencing in a cohort of 11 patients who developed angioedema following alteplase administration. Rather than identifying a shared pathogenic variant, we observed distinct yet convergent patterns of genetic vulnerability, allowing patients to be grouped according to dominant, but overlapping, biological axes. These included alterations affecting bradykinin regulation (e.g., ACE, SERPING1, XPNPEP2), endothelial structure and hemostasis (e.g., VWF, COL4A1), neurovascular and calcium signaling (e.g., SCN10A, RYR1), and vascular repair or remodeling pathways (e.g., PSEN2, BRCA2). Notably, many of the identified variants were classified as Variant of Uncertain Significance (VUS) or likely benign significance in isolation. However, when considered within an integrated, pathway-based framework, these variants can be interpreted as capable of contributing cumulatively to system level fragility, a phenomenon best described as "contextual pathogenicity". Under the acute biochemical and proteolytic stress imposed by thrombolysis, this reduced physiological reserve may allow otherwise compensated vulnerabilities to become clinically manifest. Together, these findings support a model in which severe alteplase-associated angioedema appears as an emergent property of interacting genetic networks, rather than a monogenic disorder. This systems level perspective underscores the limitations of gene centric interpretation for adverse drug reactions and highlights the potential value of pathway informed, multi-genic approaches to risk stratification. Such frameworks may ultimately contribute to safer, more personalized thrombolytic decision, while providing a conceptual foundation for future functional and translational studies.
Chronic Lymphocytic Leukemia Presenting with Acquired Angioedema.
Acquired angio-oedema as a mimicker of drug allergy.
A woman in her 50s with a history of splenic marginal zone lymphoma developed severe angio-oedema after a scaling and root planing dental procedure. She was seen by multiple physicians who treated her for presumed local anaesthetic allergy and subsequently referred her to an academic allergy centre for specialised drug allergy testing. On evaluation, her clinical history was suggestive of acquired angio-oedema, which was confirmed by bloodwork. This case report underscores key clinical pearls that should prompt clinicians to consider a diagnosis of acquired angio-oedema, a rare bradykinin-mediated disease which can present with acute life-threatening angio-oedema and for which a clinical diagnosis is critical to selecting the appropriate acute therapy. The case also highlights the utility of a complement C4 test when patients present acutely with angio-oedema of unknown aetiology, especially in the emergency department.
Recurrent Airway Swelling in a Patient With Lymphoproliferative Disorder: A Diagnostic Challenge Between Anaphylaxis and Acquired Angioedema.
Distinguishing between anaphylaxis and bradykinin-mediated angioedema can be difficult in the acute setting, particularly when airway compromise dominates the presentation. Recognition is crucial, as management strategies differ substantially. An 84-year-old woman presented to the Emergency Department in Preston with rapidly progressive swelling of the lips, tongue, and neck, accompanied by dysphagia and a hoarse voice. She had self-administered multiple doses of an adrenaline autoinjector before arrival. On examination, she was hemodynamically stable and afebrile, with marked tongue and floor-of-mouth edema but no urticaria or pruritus. Considering refractory anaphylaxis, she received intravenous dexamethasone, an adrenaline nebulizer, and an adrenaline infusion. ENT and ICU teams were involved; flexible nasendoscopy confirmed edema of the floor of the mouth and arytenoids, with mild epiglottic involvement but an adequate glottic opening. This episode followed several similar admissions since 2022, each previously treated as anaphylaxis with limited response to adrenaline. Further investigation revealed normal mast-cell tryptase (6.1 µg/L) and persistently low C4 (<0.03 g/L) with normal C3 (1.29 g/L), findings consistent with C1-esterase inhibitor (C1-INH) deficiency. Given her history of CD5-negative low-grade B-cell lymphoproliferative disorder, a diagnosis of acquired angioedema was made following multidisciplinary review, and treatment with icatibant was initiated. This case highlights the importance of considering bradykinin-mediated angioedema in patients with recurrent "refractory anaphylaxis." The absence of urticaria, normal tryptase, and low complement levels should prompt evaluation for C1-INH deficiency, allowing timely, targeted management and improved outcomes.
Publicações recentes
Chronic Lymphocytic Leukemia Presenting with Acquired Angioedema.
Recurrent Airway Swelling in a Patient With Lymphoproliferative Disorder: A Diagnostic Challenge Between Anaphylaxis and Acquired Angioedema.
Unraveling angioedema: diagnostic challenges and emerging therapies.
[Acquired angioedema associated with non-Hodgkin lymphoma: a clinical case report].
Acquired Angioedema Related to Transient C1- Inhibitor Deficiency Triggered by Mycoplasma pneumoniae Infection: A Case Report.
📚 EuropePMC152 artigos no totalmostrando 183
Chronic Lymphocytic Leukemia Presenting with Acquired Angioedema.
Turkish journal of haematology : official journal of Turkish Society of HaematologyEffect of Recombinant Tissue-Type Plasminogen Activator on Peripheral Blood Mononuclear Cells of Patients With Alteplase-Associated Angioedema.
Journal of investigational allergology & clinical immunologyAlteplase and Angioedema: Can Clinical Exome Sequencing Redefine the Paradigm?
Life (Basel, Switzerland)Recurrent Airway Swelling in a Patient With Lymphoproliferative Disorder: A Diagnostic Challenge Between Anaphylaxis and Acquired Angioedema.
CureusUnraveling angioedema: diagnostic challenges and emerging therapies.
Frontiers in immunology[Acquired angioedema associated with non-Hodgkin lymphoma: a clinical case report].
Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)Acquired Angioedema Related to Transient C1- Inhibitor Deficiency Triggered by Mycoplasma pneumoniae Infection: A Case Report.
Reports (MDPI)A hidden clue behind angioedema in an elderly patient.
Oxford medical case reportsLong-term prophylactic treatment with deucrictibant for angioedema due to acquired C1-inhibitor deficiency.
The Journal of allergy and clinical immunologyBradykinin-Mediated Angioedema Induced by Drugs.
Journal of clinical medicineA Rare Case of Idiopathic Angioedema Associated With Amlodipine Use.
CureusAcquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature.
Journal of primary care & community healthExercise-Induced Angioedema, Urticaria, and Anaphylaxis-A Narrative Review.
Sports (Basel, Switzerland)Acquired Angioedema Due to C1-Esterase Inhibitor Deficiency: A Diagnostic and Therapeutic Challenge.
Journal of investigational allergology & clinical immunologyAcquired angioedema and rituximab-induced acute thrombocytopenia in splenic marginal zone lymphoma.
Acta oncologica (Stockholm, Sweden)[Angioedema Due to Acquired C1 Inhibitor Deficiency as a Clinical Manifestation of Marginal Zone Lymphoma: A Case Report].
Revista medica de ChileNatural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study.
Children (Basel, Switzerland)Atypical lymphoproliferative disorder and acquired angioedema in systemic lupus erythematosus and Sjögren's syndrome: A diagnostic challenge.
Modern rheumatology case reportsAcquired angio-oedema as a mimicker of drug allergy.
BMJ case reportsThe Impact of COVID-19 on Patients With Bradykinin-Mediated Angioedema in the ITACA Cohort.
Clinical and experimental allergy : journal of the British Society for Allergy and Clinical ImmunologyRecurrent Acquired Angioedema as a Heralding Sign of Splenic Marginal Zone Lymphoma Recurrence.
CureusWhole Exome Sequencing in Drug-Induced Angioedema Caused by Angiotensin-Converting Enzyme Inhibitors: A Pilot Study in Five Patients.
Journal of clinical medicineHereditary angioedema diagnosis evaluation score (HADES): A new clinical scoring system for predicting hereditary angioedema with C1 inhibitor deficiency.
The journal of allergy and clinical immunology. GlobalAngioedema Due to Acquired C1-Inhibitor Deficiency Without Hematological Condition: A Multicenter French Cohort Study of 34 Patients.
The journal of allergy and clinical immunology. In practiceA multi-centre UK-based survey on angioedema secondary to acquired C1 inhibitor deficiency.
Clinical and experimental immunologyThe international HAE guideline under real-life conditions: From possibilities to limits in daily life - current real-world data of 8 German angioedema centers.
Allergologie selectAngioedema as a Presenting Feature in a Patient With SLE: A Case Report.
CureusAcquired Angioedema Post COVID-19 Infection: Can SARS-Cov-2 Induce Angioedema?
CureusAngioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort.
The journal of allergy and clinical immunology. In practiceEdema of the Scrotum and Penile Shaft: An Uncommon Initial Presentation of Acquired Angioedema With Low C1-Inhibitor.
Case reports in urologyAcute Abdominal Pain as the Initial Presentation of an Acquired C1 Inhibitor Deficiency.
GE Portuguese journal of gastroenterologyAltered levels of phospholipases C, diacylglycerols, endocannabinoids, and N-acylethanolamines in patients with hereditary angioedema due to FXII mutation.
AllergyPredictive model to differentiate chronic histaminergic angioedema and chronic spontaneous urticaria with angioedema.
The journal of allergy and clinical immunology. GlobalClinical features and potential markers of disease in idiopathic non-histaminergic angioedema, a real-life study.
Immunologic researchExtremely Late Diagnosis of Hereditary Angioedema Type I in an Elderly Female.
CureusAcquired angioedema as a late-onset complication after cord blood transplantation: a subtype of chronic graft-versus-host disease.
QJM : monthly journal of the Association of PhysiciansDeucrictibant for angioedema due to acquired C1-inhibitor deficiency: A randomized-controlled trial.
The Journal of allergy and clinical immunologyAcquired Angioedema Associated with Lymphoproliferative Disorders.
Case reports in oncologyVision loss due to atypical bilateral edema of the optic nerve in a patient with hereditary angioedema: A case report.
European journal of ophthalmologyAcquired Angioedema and Chronic Lymphocytic Leukemia: Unraveling the Complex Interplay and Addressing Refractory Cases.
CureusA Case of Acquired Angioedema Leading to the Diagnosis of Systemic Lupus Erythematosus.
CureusPseudo-Allergies in the Emergency Department: A Common Misdiagnosis of Hypersensitivity Type 1 Allergic Reaction.
CureusPathophysiology of bradykinin and histamine mediated angioedema.
Frontiers in allergyA Retrospective Analysis of Long-Term Prophylaxis with Berotralstat in Patients with Hereditary Angioedema and Acquired C1-Inhibitor Deficiency-Real-World Data.
Clinical reviews in allergy & immunologyEfficacy and Safety of Rituximab-Based Treatments in Angioedema With Acquired C1-Inhibitor Deficiency.
The journal of allergy and clinical immunology. In practiceAcquired Non-histaminergic Angioedema With C1q Autoantibody and Urticaria: A Case Report.
CureusAcquired Angioedema Due to C1-Inhibitor Deficiency (AAE-C1-INH)-A Bicenter Retrospective Study on Diagnosis, Course, and Therapy.
The journal of allergy and clinical immunology. In practiceAn unusual case of acquired angioedema associated with monoclonal gammopathies of uncertain significance.
Allergologia et immunopathologiaOver diagnosis of bradykinin angioedema in patients treated with angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers.
The World Allergy Organization journalLife-threatening Idiopathic Nonhistaminergic Acquired Angioedema With Response to Lanadelumab.
Journal of investigational allergology & clinical immunologyAngioedema in the Absence of C1 Esterase Inhibitor Deficiency in a Young Patient With Anti-dsDNA Negative Lupus Nephritis.
CureusThe impact of puberty on the onset, frequency, location, and severity of attacks in hereditary angioedema due to C1-inhibitor deficiency: A survey from the Italian Network for Hereditary and Acquired Angioedema (ITACA).
Frontiers in pediatricsRituximab in the Treatment of Acquired Angioedema Secondary to Marginal Zone Lymphoma of the Spleen.
CureusMulticentric Observational Study on Safety and Tolerability of COVID-19 Vaccines in Patients with Angioedema with C1 Inhibitor Deficiency: Data from Italian Network on Hereditary and Acquired Angioedema (ITACA).
VaccinesAcquired Angioedema in Selected Neoplastic Diseases.
Medicina (Kaunas, Lithuania)Acquired angioedema late in the course of nivolumab treatment.
Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners[Rare manifestations of monoclonal gammopathies: About two clinical cases and literature review].
La Revue de medecine interneC1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency.
Orphanet journal of rare diseasesThe validity and reliability of a Thai version of the Angioedema Control Test: Which recall period is preferable?
Asian Pacific journal of allergy and immunologyAngioedema as a Rare Presentation of Systemic Lupus Erythematosus.
CureusLong term follow-up of complement parameters to improve the management of acquired angioedema due to C1-inhibitor deficiency.
HeliyonAcquired Angioedema Acute Attack After Administration of the Moderna COVID-19 (mRNA-1273) Vaccine.
Archivos de bronconeumologiaPregnancy in women with Hereditary Angioedema due to C1-inhibitor deficiency: Results from the ITACA cohort study on outcome of mothers and children with in utero exposure to plasma-derived C1-inhibitor.
Frontiers in medicineAcquired angioedema: an unusual presentation of haematological malignancy.
BMJ case reportsLongitudinal experience with treatment of acquired angioedema using tranexamic acid.
Allergy and asthma proceedingsIsolated angioedema: A review of classification and update on management.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & ImmunologyProfile of serious angioedema requiring an urgent advice from a national reference call center.
MedicineA case of acquired angioedema.
JAAPA : official journal of the American Academy of Physician AssistantsPractical Management of New-Onset Urticaria and Angioedema Presenting in Primary Care, Urgent Care, and the Emergency Department.
The Permanente journalAcquired Angioedema due to C1-Inhibitor Deficiency: A Challenging Condition.
International archives of allergy and immunologyAngioedema-like presentation as the presenting finding of juvenile myositis and juvenile dermatomyositis in 2 patients.
International journal of rheumatic diseasesAttenuated androgen discontinuation in patients with hereditary angioedema: a commented case series.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyThe characteristics of upper airway edema in hereditary and acquired angioedema with C1-inhibitor deficiency.
Clinical and translational allergyAngioedema Without Wheals: Challenges in Laboratorial Diagnosis.
Frontiers in immunologyHereditary Angioedema: Diagnostic Algorithm and Current Treatment Concepts.
Indian dermatology online journalMalignancy and immune disorders in patients with hereditary angioedema.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyAnesthetic management of a patient with acquired angioedema submitted to broncofibroscopy: a case report.
Brazilian journal of anesthesiology (Elsevier)Angioedema Due to Acquired Deficiency of C1-Inhibitor: A Cohort Study in Spain and a Comparison With Other Series.
The journal of allergy and clinical immunology. In practicePhysicochemical and Biological Characterization of rhC1INH Expressed in CHO Cells.
Pharmaceuticals (Basel, Switzerland)Resolution of Laryngeal Oedema in a Patient with Acquired C1-Inhibitor Deficiency. a Case Report.
Journal of critical care medicine (Universitatea de Medicina si Farmacie din Targu-Mures)[Diagnosis of hereditary angioedema after thirty years of clinical manifestations].
Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)Acquired angioedema in B cell lymphoproliferative disease: A retrospective case series.
Clinical and experimental immunologyWhere we are with acquired angioedema due to C1 inhibitor deficiency: A systematic literature review.
Clinical immunology (Orlando, Fla.)National audit of a hereditary and acquired angioedema cohort in New Zealand.
Internal medicine journalEpisodic Angioedema with Hypereosinophilia (Gleich's Syndrome): A Case Report and Extensive Review of the Literature.
Journal of clinical medicineHereditary angioedema: how to approach it at the emergency department?
Einstein (Sao Paulo, Brazil)How Angioedema Quality of Life Questionnaire Can Help Physicians in Treating C1-Inhibitor Deficiency Patients?
Clinical reviews in allergy & immunologyAngioedema without wheals: a clinical update.
Balkan medical journalEffectiveness of C1-INH therapy in angiotensin converting enzyme inhibitor induced angioedema.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyEfficacy of lanadelumab in acquired angioedema with C1-inhibitor deficiency.
The journal of allergy and clinical immunology. In practiceLeveraging Genetics for Hereditary Angioedema: A Road Map to Precision Medicine.
Clinical reviews in allergy & immunologyAcquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients.
International archives of allergy and immunologyThe Panorama of Primary Angioedema in the Brazilian Population.
The journal of allergy and clinical immunology. In practiceA follow-up survey of patients with acquired angioedema due to C1-inhibitor deficiency.
Journal of internal medicineDeciphering the Genetics of Primary Angioedema with Normal Levels of C1 Inhibitor.
Journal of clinical medicineAngioedema. Interdisciplinary diagnostic and therapeutic recommendations of the Polish Dermatological Society (PTD) and Polish Society of Allergology (PTA).
Postepy dermatologii i alergologiiThe Importance of Complement Testing in Acquired Angioedema Related to Angiotensin-Converting Enzyme Inhibitors.
The journal of allergy and clinical immunology. In practiceAcquired Angioedema due to C1 Inhibitor Deficiency Preceding Splenic Marginal Zone Lymphoma: Further Insights from Clinical Practice.
International archives of allergy and immunologyRefractory acquired angioedema in chronic lymphocytic leukemia.
Leukemia & lymphomaAn Unusual Cause of Intermittent Abdominal Pain.
GastroenterologyIn Vitro Modeling of Bradykinin-Mediated Angioedema States.
Pharmaceuticals (Basel, Switzerland)Measurement of Bradykinin Formation and Degradation in Blood Plasma: Relevance for Acquired Angioedema Associated With Angiotensin Converting Enzyme Inhibition and for Hereditary Angioedema Due to Factor XII or Plasminogen Gene Variants.
Frontiers in medicine[Acquired angioedema due to C1-inhibitor deficiency: CREAK recommendations for diagnosis and treatment].
La Revue de medecine interneGastrointestinal manifestations of angioedema: a potential area of misdiagnosis.
European journal of gastroenterology & hepatologySuccessful Use of Recombinant Human C1-INH in a Patient with Acquired Angioedema due to C1 Inhibitor Deficiency and an Unusually High Titer of Anti-C1-Inhibitor Autoantibodies.
Journal of investigational allergology & clinical immunologyIdiopathic nonhistaminergic acquired angioedema in a patient with coronavirus disease 2019.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & ImmunologyAcquired angioedema induced by angiotensin-converting enzyme inhibitors - experience of a hospital-based allergy center.
Experimental and therapeutic medicineAcquired Angioedema as the Presenting Feature of a JAK2-Positive Essential Thrombocytosis.
JCO oncology practiceNailfold Videocapillaroscopy Findings in Bradykinin-Mediated Angioedema.
Journal of investigational allergology & clinical immunologyAcquired C1-inhibitor deficiency due to splenic marginal zone lymhoma: Case Report.
Acta clinica BelgicaAn Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female.
Journal of investigative medicine high impact case reportsHereditary Angioedema: An Updated Experience with Patients with Angioedema in Puerto Rico.
Puerto Rico health sciences journalAngioedema as a systemic disease.
Clinics in dermatologyAcquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaHereditary and acquired angioedema.
Allergy and asthma proceedingsA Case of Acquired Angioedema with Low C1 Inhibitor (C1-INH) Associated with Splenic Marginal Zone Lymphoma.
The American journal of case reportsSystematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.
Frontiers in immunologySerum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
Clinical and experimental immunologyAnaesthetic management of patients with pre-existing allergic conditions: a narrative review.
British journal of anaesthesiaAngioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate.
Orphanet journal of rare diseasesIdentification of the recently described plasminogen gene mutation p.Lys330Glu in a family from Northern Germany with hereditary angioedema.
Clinical and translational allergyIsolated angioedema: An overview of clinical features and etiology.
Experimental and therapeutic medicineMelkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyDiagnosis of solitary extramedullary plasmacytoma located in the nasopharynx in a patient with acquired angioedema.
Postepy dermatologii i alergologiiLong-term effects upon rituximab treatment of acquired angioedema due to C1-inhibitor deficiency.
AllergyNine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyDeletions in SERPING1 Lead to Lower C1 Inhibitor Function: Lower C1 Inhibitor Function Can Predict Disease Severity.
International archives of allergy and immunologyUrticaria and angioedema.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyOmalizumab for Idiopathic Nonhistaminergic Angioedema: Evidence for Efficacy in 2 Patients.
Case reports in immunologyReal-world outcomes in hereditary angioedema: first experience from the Icatibant Outcome Survey in the United Kingdom.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologySplenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization.
Medical oncology (Northwood, London, England)Angioedema Due to Bradykinin Dysregulation.
The journal of allergy and clinical immunology. In practiceAcquired angioedema in juvenile systemic lupus erythematosus: case-based review.
Rheumatology internationalCase report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levels.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology[Tranexamic acid as first-line emergency treatment for episodes of bradykinin-mediated angioedema induced by ACE inhibitors].
La Revue de medecine interneQuantification of human complement C2 protein using an automated turbidimetric immunoassay.
Clinical chemistry and laboratory medicineEpidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies.
Orphanet journal of rare diseasesIdiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema.
The journal of allergy and clinical immunology. In practiceAtypical presentation of acquired angioedema.
CutisRefractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency.
Case reports in hematologyAcquired Angioedema With Anti-C1-inhibitor Autoantibodies During Assisted Reproduction Techniques.
Journal of investigational allergology & clinical immunologyA Rare Cause of Recurrent Abdominal Pain and Diarrhea.
GastroenterologyHereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes.
International archives of allergy and immunologyManagement of pediatric 'cannot intubate, cannot oxygenate'.
Acute medicine & surgeryIdiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials.
Clinical and translational allergyAcquired C1 Inhibitor Deficiency.
Immunology and allergy clinics of North AmericaAngioedema: Perioperative management.
SAGE open medical case reportsDiagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency.
The journal of allergy and clinical immunology. In practiceRecurrent angioedema associated with pharmacological inhibition of dipeptidyl peptidase IV.
BMJ case reportsComparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.
Clinical and experimental immunologyIdiopathic Non-histaminergic Angioedema: Successful Treatment with Omalizumab in Five Patients.
Journal of clinical immunologyOverview of Laboratory Testing and Clinical Presentations of Complement Deficiencies and Dysregulation.
Advances in clinical chemistryPerioperative course in patients with hereditary or acquired angioedema.
Journal of clinical anesthesiaA nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients.
MedicineMonoclonal gammopathy of cutaneous significance: review of a relevant concept.
Journal of the European Academy of Dermatology and Venereology : JEADVNovelties in the Diagnosis and Treatment of Angioedema.
Journal of investigational allergology & clinical immunologyShortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema.
International archives of allergy and immunologyClinical presentation, pathophysiology, diagnosis, and treatment of acquired and hereditary angioedema: Exploring state-of-the-art therapies in RI.
Rhode Island medical journal (2013)"Nuts and Bolts" of Laboratory Evaluation of Angioedema.
Clinical reviews in allergy & immunologyAngioedema Phenotypes: Disease Expression and Classification.
Clinical reviews in allergy & immunologyA case of acquired angioedema possibly associated with adenocarcinoma of the colon.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & ImmunologyHereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment.
Annals of medicineRituximab in a patient with splenic marginal zone lymphoma and acquired angioedema.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & ImmunologyIdiopathic histaminergic angioedema without wheals: a case series of 31 patients.
Clinical and experimental immunologyC1 Inhibitor as a glycoprotein: The influence of polysaccharides on its function and autoantibody target.
Molecular immunologyBradykinin-mediated angioedema.
Polskie Archiwum Medycyny Wewnetrznej'Epinephrine-resistant' angioedema.
BMJ case reportsNanofiltrated C1-esterase-inhibitor in the prophylactic treatment of bradykinin-mediated angioedema.
TransfusionHigh prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency.
British journal of haematology[Acquired angioedema – clinical characteristic of the patients diagnosed in 2012-2016 with acquired C1 inhibitor deficiency].
Przeglad lekarskiAngioedema Related to Angiotensin-Converting Enzyme Inhibitors: Attack Severity, Treatment, and Hospital Admission in a Prospective Multicenter Study.
MedicineAssociation between thyroid autoimmunity and recurrent angioedema in children.
Allergy and asthma proceedingsNon-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?
The Netherlands journal of medicine[Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebieteELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients.
Journal of immunological methodsThe Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity.
Clinical chemistry and laboratory medicineManagement of acute attacks of hereditary angioedema: role of ecallantide.
Journal of blood medicineRecurrent intestinal obstruction with acquired angio-oedema, due to C1-esterase inhibitor deficiency.
The Journal of the Association of Physicians of IndiaFacial angioedema and systemic lupus erythematosus: case report.
Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial SurgeonsCharacterization of patients with angioedema without wheals: the importance of F12 gene screening.
Clinical immunology (Orlando, Fla.)Complement system in dermatological diseases - fire under the skin.
Frontiers in medicineTreatment of ACEI-related angioedema with icatibant: a case series.
Internal and emergency medicineLesson of the month 2: The limitations of steroid therapy in bradykinin-mediated angioedema attacks.
Clinical medicine (London, England)Associações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Angioedema adquirido.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Angioedema adquirido
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Effect of Recombinant Tissue-Type Plasminogen Activator on Peripheral Blood Mononuclear Cells of Patients With Alteplase-Associated Angioedema.
- Alteplase and Angioedema: Can Clinical Exome Sequencing Redefine the Paradigm?
- Chronic Lymphocytic Leukemia Presenting with Acquired Angioedema.Turkish journal of haematology : official journal of Turkish Society of Haematology· 2026· PMID 41867000mais citado
- Acquired angio-oedema as a mimicker of drug allergy.
- Recurrent Airway Swelling in a Patient With Lymphoproliferative Disorder: A Diagnostic Challenge Between Anaphylaxis and Acquired Angioedema.
- Unraveling angioedema: diagnostic challenges and emerging therapies.
- [Acquired angioedema associated with non-Hodgkin lymphoma: a clinical case report].
- Acquired Angioedema Related to Transient C1- Inhibitor Deficiency Triggered by Mycoplasma pneumoniae Infection: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:91385(Orphanet)
- MONDO:0019624(MONDO)
- GARD:8605(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q4674768(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
