Acquired angioedema is a rare, nonhereditary, bradykinin-mediated angioedema syndrome caused by acquired C1 inhibitor deficiency or dysfunction. It typically begins in adulthood, lacks a family history of hereditary angioedema, and is strongly associated with B-cell lymphoproliferative disorders, monoclonal gammopathy, and anti-C1-INH autoantibodies.
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name: Acquired Angioedema
creation_date: "2026-05-10T16:46:20Z"
updated_date: "2026-05-10T17:57:06Z"
category: Complex
description: >-
Acquired angioedema is a rare, nonhereditary, bradykinin-mediated angioedema
syndrome caused by acquired C1 inhibitor deficiency or dysfunction. It
typically begins in adulthood, lacks a family history of hereditary
angioedema, and is strongly associated with B-cell lymphoproliferative
disorders, monoclonal gammopathy, and anti-C1-INH autoantibodies.
disease_term:
preferred_term: acquired angioedema
term:
id: MONDO:0019624
label: acquired angioedema
synonyms:
- AAE
- AAE-C1-INH
- acquired angioedema due to C1-inhibitor deficiency
- acquired C1 inhibitor deficiency
- acquired angioneurotic edema
- acquired bradykinin-induced angioedema
parents:
- Angioedema
- Complement Disorder
prevalence:
- population: Czech Republic
percentage: "1:760,000"
notes: >-
Nationwide retrospective ascertainment estimated acquired angioedema with
C1-INH deficiency as an ultra-rare condition in the Czech Republic.
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The prevalence of AAE-C1-INH in the Czech Republic is about 1:760,000.
This rare condition occurs in approximately 8% of the patients with
angioedema with C1 inhibitor deficiency.
explanation: >-
The national cohort abstract provides the prevalence estimate and
proportion among C1-INH-deficiency angioedema cases.
progression:
- phase: Adult onset episodic attacks
age_range: Adult onset, typically after 40 years
notes: >-
AAE-C1-INH usually starts in late adulthood and follows a recurrent attack
pattern rather than a progressive fixed swelling state.
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The median age of the symptom onset was 59.5 years, and the median
diagnosis delay was 1 year.
explanation: >-
This supports typical late-adult onset and a measurable diagnostic delay
in the Czech nationwide cohort.
pathophysiology:
- name: B-cell Disorder-Associated C1-INH Consumption
description: >-
B-cell lymphoproliferative disease, monoclonal gammopathy, or related
antibody-producing states can drive acquired consumption or neutralization
of C1 inhibitor and complement components.
cell_types:
- preferred_term: B cell
term:
id: CL:0000236
label: B cell
- preferred_term: plasma cell
term:
id: CL:0000786
label: plasma cell
biological_processes:
- preferred_term: complement activation, classical pathway
term:
id: GO:0006958
label: complement activation, classical pathway
modifier: INCREASED
evidence:
- reference: PMID:30866985
reference_title: "Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The following underlying disorders were present: monoclonal gammopathy of
undetermined significance (47.7%), non-Hodgkin lymphoma (27.3%),
anti-C1-INH autoantibodies alone (11.4%), and other conditions (4.5%).
explanation: >-
This referral cohort supports B-cell and autoantibody-associated
conditions as common upstream contexts for AAE-C1-INH.
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Lymphoid malignancy was identified in 9 patients (64%), monoclonal
gammopathy of uncertain significance in 3 (21%), and in 1 patient
autoimmune disease (ulcerative colitis) was considered causative (7%).
explanation: >-
The national cohort independently supports lymphoid malignancy, MGUS, and
autoimmune disease as frequent associated upstream conditions.
downstream:
- target: Acquired C1 Inhibitor Deficiency
causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
intermediate_mechanisms:
- C1-INH consumption
- autoantibody-mediated C1-INH inactivation
- name: Anti-C1-INH Immune Complex Formation
description: >-
Anti-C1-INH autoantibodies can circulate as free antibodies or as
C1-INH/anti-C1-INH complexes, providing a mechanism for functional C1-INH
loss and a diagnostic marker in some patients.
cell_types:
- preferred_term: plasma cell
term:
id: CL:0000786
label: plasma cell
biological_processes:
- preferred_term: complement activation, classical pathway
term:
id: GO:0006958
label: complement activation, classical pathway
modifier: INCREASED
evidence:
- reference: PMID:31397881
reference_title: Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
C1INH-antiC1INHAb complexes were found in 18 of 20 of the AAE cases,
regardless of the presence or absence of detectable free anti-C1INHAbs.
explanation: >-
This human cohort demonstrates that anti-C1-INH antibodies and immune
complexes are frequent in AAE-C1-INH.
- reference: PMID:36726161
reference_title: C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Free circulating and complex antibodies are in a dynamically changing
equilibrium.
explanation: >-
This supports a dynamic antibody/immune-complex mechanism rather than a
static single antibody measurement.
downstream:
- target: Acquired C1 Inhibitor Deficiency
causal_link_type: DIRECT
- name: Acquired C1 Inhibitor Deficiency
description: >-
Secondary quantitative or functional C1 inhibitor deficiency reduces
physiologic inhibition of complement and kallikrein-kinin/contact pathways.
biological_processes:
- preferred_term: complement activation
term:
id: GO:0006956
label: complement activation
modifier: INCREASED
evidence:
- reference: PMID:31397881
reference_title: Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Acquired angioedema due to C1-inhibitor (C1INH) deficiency (AAE) is
caused by secondary C1INH deficiency leading to bradykinin-mediated
angioedema episodes.
explanation: >-
The abstract directly states the acquired C1-INH deficiency mechanism and
connects it to bradykinin-mediated attacks.
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The inclusion criteria involved recurrent episodes of angioedema with the
first manifestation at or after the age of 40, negative family history of
angioedema, and C1 inhibitor function 50% or less.
explanation: >-
This cohort definition supports low C1-INH function plus late onset and
negative family history as defining features of acquired disease.
downstream:
- target: Bradykinin Excess
causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
intermediate_mechanisms:
- kallikrein-kinin system dysregulation
- contact system activation
- name: Bradykinin Excess
description: >-
Loss of C1-INH control permits excess bradykinin generation, the proximal
mediator of nonhistaminergic angioedema attacks.
biological_processes:
- preferred_term: bradykinin biosynthetic process
term:
id: GO:0002936
label: bradykinin biosynthetic process
modifier: INCREASED
chemical_entities:
- preferred_term: bradykinin
term:
id: CHEBI:3165
label: bradykinin
evidence:
- reference: PMID:34956216
reference_title: "Angioedema Without Wheals: Challenges in Laboratorial Diagnosis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Although bradykinin-mediated disease results mainly from disturbance in
the kallikrein-kinin system, traditionally complement evaluation has been
used for diagnosis.
explanation: >-
This diagnostic review supports kallikrein-kinin disturbance as the
bradykinin-mediated disease mechanism.
downstream:
- target: Bradykinin-Driven Vascular Permeability
causal_link_type: DIRECT
- name: Bradykinin-Driven Vascular Permeability
description: >-
Excess bradykinin signaling increases endothelial permeability and produces
localized, transient plasma extravasation in skin, bowel, and upper-airway
tissues.
cell_types:
- preferred_term: endothelial cell
term:
id: CL:0000115
label: endothelial cell
biological_processes:
- preferred_term: positive regulation of vascular permeability
term:
id: GO:0043117
label: positive regulation of vascular permeability
modifier: INCREASED
locations:
- preferred_term: face
term:
id: UBERON:0001456
label: face
- preferred_term: larynx
term:
id: UBERON:0001737
label: larynx
- preferred_term: digestive tract
term:
id: UBERON:0001555
label: digestive tract
evidence:
- reference: PMID:34956216
reference_title: "Angioedema Without Wheals: Challenges in Laboratorial Diagnosis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
They present with subcutaneous and mucosal swellings, affecting
extremities, face, genitals, bowels, and upper airways.
explanation: >-
This supports the anatomic distribution of bradykinin-mediated
angioedema attacks.
phenotypes:
- category: Dermatologic
name: Recurrent Angioedema
description: >-
Recurrent nonurticarial swelling affects subcutaneous or mucosal tissues and
resolves between attacks.
phenotype_term:
preferred_term: angioedema
term:
id: HP:0100665
label: Angioedema
temporality: RECURRENT
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The inclusion criteria involved recurrent episodes of angioedema with the
first manifestation at or after the age of 40, negative family history of
angioedema, and C1 inhibitor function 50% or less.
explanation: >-
The cohort inclusion criteria directly support recurrent angioedema as
the central clinical phenotype.
- category: Craniofacial
name: Facial Edema
description: >-
Facial swelling is a prominent manifestation of acquired C1-INH deficiency
attacks.
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: facial edema
term:
id: HP:0000282
label: Facial edema
temporality: RECURRENT
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The most common clinical manifestation was facial edema (100%) and upper
airway swelling (85.7%).
explanation: >-
The national cohort identifies facial edema as the most common clinical
manifestation.
- category: Respiratory
name: Laryngeal Edema
description: >-
Upper-airway or laryngeal swelling can cause airway compromise and is the
main life-threatening manifestation.
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: laryngeal edema
term:
id: HP:0012027
label: Laryngeal edema
temporality: RECURRENT
severity: SEVERE
evidence:
- reference: PMID:30866985
reference_title: "Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Acquired angioedema due to C1-inhibitor (C1-INH) deficiency
(AAE-C1-INH) is a serious condition that may result in life-threatening
asphyxiation due to laryngeal edema.
explanation: >-
This cohort abstract directly supports life-threatening laryngeal edema
in AAE-C1-INH.
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The most common clinical manifestation was facial edema (100%) and upper
airway swelling (85.7%).
explanation: >-
The national cohort reports upper-airway swelling in 85.7% of patients,
supporting the VERY_FREQUENT frequency assignment.
- category: Gastrointestinal
name: Bowel Angioedema with Episodic Abdominal Pain
description: >-
Bowel wall swelling can produce episodic abdominal pain during attacks,
with abdominal involvement reported in a majority of patients in a large
acquired C1-INH deficiency cohort.
frequency: FREQUENT
phenotype_term:
preferred_term: episodic abdominal pain
term:
id: HP:0002574
label: Episodic abdominal pain
evidence:
- reference: PMID:28284781
reference_title: "Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: "followed by abdomen (N = 51"
explanation: >-
The Milan cohort reports abdominal involvement in 66% of C1-INH-AAE
patients, supporting abdominal or bowel angioedema as a frequent
manifestation.
- category: Dermatologic
name: Peripheral Edema
description: >-
Limb or extremity swelling can occur as part of the nonurticarial
angioedema attack distribution.
frequency: FREQUENT
phenotype_term:
preferred_term: edema of extremities
term:
id: HP:0000969
label: Edema
temporality: RECURRENT
evidence:
- reference: PMID:28284781
reference_title: "Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: "peripheries (N = 50"
explanation: >-
The cohort reports peripheral involvement in 65% of patients. Local HPO
search did not identify a more specific extremity edema term, so the
broad edema term is retained with a specific preferred term.
biochemical:
- name: Low C1 Inhibitor Function
presence: DECREASED
context: >-
Low C1-INH function is part of the diagnostic definition of AAE-C1-INH and
distinguishes the syndrome from angioedema with normal C1-INH.
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The inclusion criteria involved recurrent episodes of angioedema with the
first manifestation at or after the age of 40, negative family history of
angioedema, and C1 inhibitor function 50% or less.
explanation: >-
The cohort required low C1-INH function, supporting this as a core
biochemical abnormality.
- name: Low C4
presence: DECREASED
context: >-
Low C4 is part of the complement-consumption pattern used with C1-INH
antigen/function and clinical context to support acquired C1-INH deficiency.
evidence:
- reference: PMID:28284781
reference_title: "Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Diagnostic criteria included history of recurrent angioedema without
wheals; decreased C1-INH antigen levels and/or functional activity of
C1-INH and C4 antigen less than 50% of normal; late symptom onset (>40
years); no family history of angioedema and C1-INH deficiency.
explanation: >-
The cohort's diagnostic criteria explicitly include C4 antigen below 50%
of normal together with C1-INH abnormalities and adult-onset recurrent
angioedema.
- name: Low or Undetectable C1q
presence: DECREASED
context: >-
Low C1q supports acquired C1-INH deficiency in the right clinical setting,
but is not specific by itself.
evidence:
- reference: PMID:31397881
reference_title: Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
When free antiC1INHAbs and malignant tumors are not detectable, diagnosis
relies on the finding of low C1INH levels and/or function, lack of family
history and SERPING1 mutations, age at onset and low or undetectable C1q
levels, none of which is specific for AAE.
explanation: >-
This supports low C1q as part of the diagnostic biochemical pattern while
preserving the authors' caveat that it is not specific alone.
- name: C1-INH-Anti-C1-INH Immune Complexes
presence: PRESENT
context: >-
C1-INH/anti-C1-INH immune complexes can be detected even when free
anti-C1-INH antibodies are not measurable.
evidence:
- reference: PMID:31397881
reference_title: Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Of note, nine of 20 patients showed negative free antiC1INHabs, but
positive C1INH-antiC1INHAb complexes in their first measurement.
explanation: >-
This supports immune-complex detection as a biochemical feature that can
improve diagnostic yield.
diagnosis:
- name: Complement and C1-INH Functional Testing
description: >-
Diagnosis combines recurrent angioedema without family history with
complement/C1-INH testing, especially C1-INH function and levels, C4, C1q,
and evaluation for hereditary angioedema or histaminergic mimics.
results: >-
Low C1-INH function, low C1-INH level, low C4, and/or low C1q, with adult
onset and absence of family history or SERPING1 mutation, supports acquired
C1-INH deficiency.
evidence:
- reference: PMID:34956216
reference_title: "Angioedema Without Wheals: Challenges in Laboratorial Diagnosis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Diagnosis is established by nephelometry, turbidimetry, or radial
immunodiffusion for quantitative measurement of C1 inhibitor, and
chromogenic assay or ELISA has been used for functional C1-INH analysis.
explanation: >-
This diagnostic review identifies the assays used for quantitative and
functional C1-INH analysis.
- reference: PMID:31397881
reference_title: Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
When free antiC1INHAbs and malignant tumors are not detectable, diagnosis
relies on the finding of low C1INH levels and/or function, lack of family
history and SERPING1 mutations, age at onset and low or undetectable C1q
levels, none of which is specific for AAE.
explanation: >-
This supports the diagnostic combination and explicitly cautions that no
single component is specific on its own.
- name: C1-INH Autoantibody and Immune-Complex Testing
description: >-
Testing for free anti-C1-INH antibodies and C1-INH/anti-C1-INH complexes can
support diagnosis and monitoring, especially when free antibodies are not
detectable.
results: >-
Presence of free anti-C1-INH antibodies or C1-INH/anti-C1-INH complexes
supports AAE-C1-INH in the appropriate clinical and complement context.
evidence:
- reference: PMID:36726161
reference_title: C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Measurement of CAC is recommended to be done parallelly with C1-INH-Ab,
so as to detect both free and bound antibodies.
explanation: >-
This recent cohort supports parallel measurement of free antibodies and
C1-INH/anti-C1-INH antibody complexes.
treatments:
- name: Plasma-Derived or Recombinant C1 Inhibitor Concentrate
description: >-
C1-INH replacement is used for acute attacks and acts upstream by replacing
deficient inhibitor activity.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
target_mechanisms:
- target: Acquired C1 Inhibitor Deficiency
treatment_effect: RESTORES
description: >-
Exogenous C1-INH replacement targets the deficient inhibitor state that
drives bradykinin-mediated attacks.
evidence:
- reference: PMID:30866985
reference_title: "Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
A total of 3553 (97.7%) of the 3636 attacks were effectively treated with
pdC1-INH as assessed by the patient.
explanation: >-
This large attack-level cohort supports plasma-derived C1-INH as highly
effective on-demand therapy.
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
All patients responded to the acute attack treatment with icatibant and
plasma-derived or recombinant C1 inhibitor concentrate.
explanation: >-
The national cohort supports acute-attack response to C1-INH concentrate,
including recombinant C1-INH use.
- name: Icatibant On-Demand Therapy
description: >-
Icatibant blocks bradykinin B2 receptor signaling and is used as on-demand
treatment for acute AAE-C1-INH attacks.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: icatibant
term:
id: CHEBI:68556
label: icatibant
target_mechanisms:
- target: Bradykinin-Driven Vascular Permeability
treatment_effect: INHIBITS
description: >-
Bradykinin receptor antagonism is intended to inhibit downstream
permeability signaling during attacks.
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
All patients responded to the acute attack treatment with icatibant and
plasma-derived or recombinant C1 inhibitor concentrate.
explanation: >-
The cohort reports acute-attack response to icatibant among treated
patients.
- name: Berotralstat Long-Term Prophylaxis
description: >-
Berotralstat, an oral kallikrein inhibitor, has limited real-world evidence
for off-label long-term prophylaxis in AAE-C1-INH.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: berotralstat
term:
id: NCIT:C169808
label: Berotralstat
target_mechanisms:
- target: Bradykinin Excess
treatment_effect: INHIBITS
description: >-
Kallikrein inhibition targets upstream bradykinin generation and is
expected to reduce recurrent attacks.
evidence:
- reference: PMID:37914894
reference_title: A Retrospective Analysis of Long-Term Prophylaxis with Berotralstat in Patients with Hereditary Angioedema and Acquired C1-Inhibitor Deficiency-Real-World Data.
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
After 6 months of treatment, a median decrease of attacks per month was
noted for HAE type I patients (3.3 to 1.5) and AAE-C1-INH patients (2.3
to 1.0).
explanation: >-
The evidence is a small retrospective real-world AAE-C1-INH subgroup, so
the prophylaxis claim is marked PARTIAL rather than definitive.
- name: Tranexamic Acid Long-Term Prophylaxis
description: >-
Tranexamic acid is an antifibrinolytic used as long-term prophylaxis in
acquired C1-INH deficiency, with cohort evidence of benefit in many treated
patients.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: tranexamic acid
term:
id: CHEBI:48669
label: tranexamic acid
target_mechanisms:
- target: Acquired C1 Inhibitor Deficiency
treatment_effect: MODULATES
description: >-
Antifibrinolytic therapy is used in acquired C1-INH deficiency to reduce
contact/fibrinolytic-system amplification that contributes to
bradykinin-mediated attacks.
evidence:
- reference: PMID:28284781
reference_title: "Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Thirty-four patients received long-term prophylaxis with tranexamic acid
(effective in 29) and 20 with androgens (effective in 8).
explanation: >-
The Milan cohort directly reports tranexamic acid prophylaxis use and
effectiveness in most treated C1-INH-AAE patients.
- name: Treat Underlying Lymphoproliferative Disease
description: >-
Evaluation and treatment of the associated lymphoid malignancy or related
disorder can reduce angioedema activity and may normalize complement
parameters.
treatment_term:
preferred_term: therapeutic procedure
term:
id: NCIT:C49236
label: Therapeutic Procedure
target_mechanisms:
- target: B-cell Disorder-Associated C1-INH Consumption
treatment_effect: INHIBITS
description: >-
Treating the upstream B-cell or lymphoid disorder can reduce C1-INH
consumption and downstream attacks.
evidence:
- reference: PMID:33472202
reference_title: "Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
In 6 of 7 patients (86%) treated for lymphoma, either a reduction in the
frequency of angioedema attacks or both angioedema symptoms' disappearance
and complement parameter normalization was observed.
explanation: >-
This supports treating the underlying lymphoid malignancy as an upstream
disease-control strategy.
clinical_trials:
- name: NCT06818474
phase: PHASE_IV
status: RECRUITING
description: >-
Open-label Phase IV study of lanadelumab 300 mg for long-term prophylaxis
in adults with acquired angioedema.
target_phenotypes:
- preferred_term: angioedema
term:
id: HP:0100665
label: Angioedema
evidence:
- reference: clinicaltrials:NCT06818474
reference_title: Lanadelumab in Long-term Prophylaxis of Acquired Angioedema
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: use of lanadelumab in patients with acquired angioedema
explanation: >-
The registry summary directly identifies lanadelumab prophylaxis in
acquired angioedema.
- name: NCT07266805
phase: PHASE_III
status: RECRUITING
description: >-
CREAATE is a randomized Phase III study evaluating oral deucrictibant XR for
prophylaxis and deucrictibant IR for on-demand treatment of AAE-C1-INH
attacks.
target_phenotypes:
- preferred_term: angioedema
term:
id: HP:0100665
label: Angioedema
evidence:
- reference: clinicaltrials:NCT07266805
reference_title: "A Phase 3, Randomized, Double-blind, Placebo-controlled, 3-Part Study to Evaluate the Efficacy and Safety of Orally Administered Deucrictibant Extended-release (XR) Tablet for Prophylaxis and Deucrictibant Immediate-release (IR) Capsule for On-demand Treatment of Angioedema Attacks in Adults With Acquired Angioedema Due to C1 Inhibitor Deficiency"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
This is a Phase 3, multicenter, 3-part study, with 2 randomized,
double-blind, placebo-controlled parts and an open-label extension part,
to evaluate the efficacy and safety of orally administered deucrictibant
XR tablet for prophylaxis, and deucrictibant IR capsule for on-demand
treatment of angioedema attacks in adult participants aged ≥ 18 years with
AAE-C1INH.
explanation: >-
The registry summary supports the Phase III study of deucrictibant for
both prophylaxis and on-demand treatment in adults with AAE-C1-INH.
datasets:
Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH; also written AAE-C1INH or C1-INH-AAE) is a rare, non-hereditary, bradykinin-mediated angioedema characterized by recurrent episodes of subcutaneous and/or submucosal swelling that may involve the face, tongue/oral cavity, gastrointestinal tract, and upper airway, with potential for fatal laryngeal edema. It is defined by acquired deficiency or dysfunction of C1 inhibitor (C1-INH) with accompanying complement abnormalities and typically occurs in adults with no family history. (bork2019angioedemadueto pages 1-2, sobotkova2021acquiredangioedemawith pages 1-2, grumach2021angioedemawithoutwheals pages 2-3)
The information in this report is derived primarily from aggregated disease-level resources (national cohort studies, referral-center cohorts, peer-reviewed reviews) and clinical trial registry records, rather than EHR-only sources. (sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto pages 1-2, NCT07266805 chunk 1)
AAE-C1-INH arises from secondary (acquired) C1-INH deficiency, resulting from (i) consumption of C1-INH and complement components (often driven by B-cell lymphoproliferation) and/or (ii) autoantibody-mediated inactivation of C1-INH. These processes lead to dysregulated activation of complement/contact systems and excess bradykinin, increasing vascular permeability and causing angioedema. (johnson2023aretrospectiveanalysis pages 1-2, bork2019angioedemadueto pages 1-2, grumach2021angioedemawithoutwheals pages 2-3)
AAE-C1-INH is strongly associated with B-cell lymphoproliferative disorders and monoclonal gammopathies, and can also be associated with autoimmune disease.
Quantitative association data from cohorts: - Czech nationwide cohort (n=14): lymphoid malignancy 64% (9/14); MGUS 21% (3/14); autoimmune disease 7% (1/14); none identified 7% (1/14). (sobotkova2021acquiredangioedemawith pages 1-2) - Mainz referral cohort (n=44): MGUS 47.7%; non-Hodgkin lymphoma 27.3%; anti-C1-INH autoantibodies alone 11.4%; no associated disorder 9.1%. (bork2019angioedemadueto pages 1-2)
Triggers reported include mechanical trauma, emotional stress, and ACE inhibitors (as potential triggers/complicating exposures in bradykinin-mediated disease contexts). (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)
No protective factors or gene–environment interaction evidence specific to AAE-C1-INH were found in the retrieved tool evidence. Given that AAE-C1-INH is typically secondary and non-hereditary, genetic susceptibility is not generally the primary driver (contrast with hereditary angioedema). (sobotkova2021acquiredangioedemawith pages 1-2, grumach2021angioedemawithoutwheals pages 2-3)
AAE-C1-INH commonly presents with recurrent non-urticarial swelling affecting facial/oropharyngeal tissues, airway, abdomen, and extremities. In a Czech nationwide series (n=14), phenotype frequencies were: - Facial edema: 100% (14/14) - Upper airway involvement: 85.7% (12/14) - Abdominal attacks: 50% (7/14) - Peripheral angioedema: 42.8% (6/14) (sobotkova2021acquiredangioedemawith pages 4-5)
Disease onset in this cohort occurred between 40–82 years (median 59.5). (sobotkova2021acquiredangioedemawith pages 4-5)
Direct AAE-C1-INH QoL data in the retrieved evidence are limited; however, a small real-world prophylaxis series that included AAE-C1-INH patients reported improvement in angioedema-specific QoL and control measures with berotralstat (see Treatment section). (johnson2023aretrospectiveanalysis pages 1-2)
Based on the above cohort phenotypes: - Angioedema: HP:0100664 - Facial swelling: HP:0000280 - Laryngeal edema / upper airway edema: HP:0011106 (laryngeal edema) / HP:0011107 (airway edema; term usage varies) - Abdominal pain (during abdominal attacks): HP:0002027 - Edema of extremities: HP:0000969
(sobotkova2021acquiredangioedemawith pages 4-5)
AAE-C1-INH is not primarily a germline genetic disorder; it is defined by an acquired deficiency/dysfunction of C1-INH rather than inherited SERPING1 mutations. In diagnostic frameworks, lack of SERPING1 mutation supports acquired disease when combined with late onset and complement patterns. (grumach2021angioedemawithoutwheals pages 2-3, caballero2022medicalalgorithmmanagement pages 2-2)
Anti–C1-INH autoantibodies may be present as free antibodies or in immune complexes, which has diagnostic implications: - In a European AAE cohort (n=20), free anti-C1INHAbs were detected in 9/20, while C1INH–antiC1INHAb complexes were detected in 18/20; notably 9/20 were negative for free antibodies but positive for complexes at first measurement. (lopezlera2019serumcomplexesbetween pages 1-2) - A Hungarian center cohort (n=19) reported 79% with an underlying disease and recommended measuring C1-INH/C1-INH antibody complexes (CAC) in parallel with free antibody testing for improved detection and monitoring. (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)
No population-level environmental or lifestyle risk factor evidence specific to AAE-C1-INH was captured in the retrieved documents.
ACE inhibitors are mentioned as potential triggers/associations in the context of bradykinin-mediated angioedema and can confound diagnosis; AAE-C1-INH has specific complement abnormalities that help distinguish it. (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)
Because many associations are B-cell/monoclonal gammopathy driven: - B cell: CL:0000236 - Plasma cell: CL:0000786
(sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto pages 1-2)
From cohort phenotype data, commonly affected sites include: - Face: UBERON:0001456 (sobotkova2021acquiredangioedemawith pages 4-5) - Upper airway/larynx: UBERON:0001737 (larynx) (sobotkova2021acquiredangioedemawith pages 4-5, bork2019angioedemadueto pages 1-2) - Gastrointestinal tract (bowel): UBERON:0001555 (digestive tract) / UBERON:0002108 (small intestine) (abdominal attacks) (sobotkova2021acquiredangioedemawith pages 4-5) - Extremities/limbs: UBERON:0002101 (limb) (sobotkova2021acquiredangioedemawith pages 4-5)
AAE-C1-INH typically has adult onset (>40 years), often late middle age, and follows an episodic course with recurrent attacks. In the Czech nationwide cohort, the median onset age was 59.5 years and diagnosis delay median 1 year. (sobotkova2021acquiredangioedemawith pages 1-2)
Treating underlying lymphoproliferative disease can reduce attack frequency and may normalize complement parameters (reported as an observation in the Czech cohort summary). (sobotkova2021acquiredangioedemawith pages 1-2)
AAE-C1-INH is acquired and therefore not inherited in a Mendelian fashion; it is differentiated from hereditary angioedema by lack of family history and lack of SERPING1 mutation in diagnostic algorithms. (grumach2021angioedemawithoutwheals pages 2-3, caballero2022medicalalgorithmmanagement pages 2-2)
Best-available prevalence estimates from retrieved sources: - Czech Republic nationwide retrospective study: prevalence ~1:760,000; AAE-C1-INH accounted for ~8% of angioedema with C1-INH deficiency in that setting. (sobotkova2021acquiredangioedemawith pages 1-2) - Literature estimates: 1:100,000–1:500,000 inhabitants. (bork2019angioedemadueto pages 1-2, lopezlera2019serumcomplexesbetween pages 1-2) - A recent real-world prophylaxis paper cites prevalence ~0.15 per 100,000. (johnson2023aretrospectiveanalysis pages 1-2)
Sex distribution in Czech cohort was 7 male / 7 female (1:1). (sobotkova2021acquiredangioedemawith pages 4-5)
A practical and widely referenced pattern for AAE-C1-INH is: - Low C4 - Low C1-INH functional activity - Low C1-INH antigen (often) - Low C1q (frequent, helpful to differentiate acquired from hereditary forms) - Anti–C1-INH autoantibodies and/or C1-INH–anti–C1-INH immune complexes in many cases
Czech cohort laboratory frequencies provide concrete performance-like data: - Low C4: 14/14 (100%) - Low C1-INH function: 14/14 (100%) - Low C1-INH antigen: 13/14 (93%) - Low C1q: 10/14 (71.4%) (sobotkova2021acquiredangioedemawith pages 4-5)
A review focused on laboratory differentiation summarizes the pattern as AAE-C1-INH having low C1-INH function and concentration, low C4, low C1q, and frequently anti–C1-INH antibodies, without SERPING1 mutation. (grumach2021angioedemawithoutwheals pages 2-3)
A key 2019 development is demonstration that immune-complex detection may be more sensitive than free antibody detection: C1INH–antiC1INHAb complexes were found in 18/20 AAE cases even when free antibodies were negative (9/20). (lopezlera2019serumcomplexesbetween pages 1-2) A 2023 Orphanet Journal paper further argues CAC measurements can aid prediction/monitoring of underlying disease and recommends parallel measurement with free antibody. (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)
(Exact LOINC codes were not available in retrieved evidence; below are standardized test concepts commonly used clinically.) - Complement C4 [Mass/volume] in Serum/Plasma (C4) - C1 esterase inhibitor [Mass/volume] in Serum/Plasma (C1-INH antigen) - C1 esterase inhibitor activity in Serum/Plasma (C1-INH function) - Complement C1q [Mass/volume] in Serum/Plasma (C1q) - Anti–C1-INH antibody (IgG/IgM) in Serum; and/or C1-INH–anti–C1-INH immune complexes (ELISA-based) (lopezlera2019serumcomplexesbetween pages 1-2, polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)
AAE-C1-INH can be life-threatening due to laryngeal edema/asphyxiation risk. (bork2019angioedemadueto pages 1-2)
Robust survival rates, mortality rates, or long-term disability estimates were not present in the retrieved tool evidence.
No therapies are universally approved specifically for AAE-C1-INH in many jurisdictions; clinical practice often uses HAE-directed therapies off-label. (johnson2023aretrospectiveanalysis pages 1-2, bork2019angioedemadueto pages 1-2)
Because AAE-C1-INH is commonly associated with lymphoproliferative disease/MGUS, evaluation and treatment of the underlying disorder is a core real-world strategy, with cohort observations of attack reduction and complement normalization after lymphoma treatment. (sobotkova2021acquiredangioedemawith pages 1-2)
Short-term procedural prophylaxis is discussed in broader angioedema management reviews (not AAE-specific in the retrieved evidence), but AAE patients are often managed analogously to HAE in practice where clinically justified. (caballero2022medicalalgorithmmanagement pages 2-2)
No evidence for naturally occurring AAE-C1-INH as a defined disease entity in other species was identified in the retrieved evidence. AAE-C1-INH is primarily a human secondary immunologic/hematologic syndrome.
The retrieved evidence did not include specific in vivo models of acquired C1-INH deficiency angioedema. Mechanistic work in bradykinin/complement biology often uses complement/contact system models, but explicit AAE-C1-INH model-organism validation was not present in the collected sources.
| Topic | Key details (quantitative where available) | Best supporting sources (with year, journal, DOI/URL) | Notes |
|---|---|---|---|
| Acquired Angioedema (AAE-C1-INH) key facts — definition | Rare, non-hereditary, bradykinin-mediated angioedema caused by acquired C1-inhibitor deficiency; clinically similar to hereditary C1-INH deficiency; may cause life-threatening laryngeal edema/asphyxiation. Typically lacks family history and SERPING1 mutation. (bork2019angioedemadueto pages 1-2, grumach2021angioedemawithoutwheals pages 2-3) | Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 1-2); Bork et al., 2019, Orphanet J Rare Dis, doi:10.1186/s13023-019-1043-3, https://doi.org/10.1186/s13023-019-1043-3 (bork2019angioedemadueto pages 1-2); Grumach et al., 2021, Front Immunol, doi:10.3389/fimmu.2021.785736, https://doi.org/10.3389/fimmu.2021.785736 (grumach2021angioedemawithoutwheals pages 2-3) | Aggregated disease-level literature, not individual EHR-derived definitions. |
| Typical onset | Adult onset, usually after age 40; Czech nationwide cohort median symptom onset 59.5 years (range 40–82). Diagnostic delay in Czech cohort: median 1 year. (sobotkova2021acquiredangioedemawith pages 1-2, sobotkova2021acquiredangioedemawith pages 4-5) | Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 1-2, sobotkova2021acquiredangioedemawith pages 4-5); Johnson et al., 2023, Clin Rev Allergy Immunol, doi:10.1007/s12016-023-08972-2, https://doi.org/10.1007/s12016-023-08972-2 (johnson2023aretrospectiveanalysis pages 1-2) | Later onset is a major clue distinguishing AAE from hereditary disease. |
| Core lab pattern | Typical pattern: low C1-INH function, low/usually low C1-INH antigen, low C4, and often low C1q. In Czech cohort: low C4 14/14 (100%), low C1-INH function 14/14 (100%), low C1-INH antigen 13/14 (93%), low C1q 10/14 (71.4%). Anti-C1-INH antibodies are frequent but not universal. (sobotkova2021acquiredangioedemawith pages 4-5, grumach2021angioedemawithoutwheals pages 2-3) | Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 4-5); Grumach et al., 2021, Front Immunol, doi:10.3389/fimmu.2021.785736, https://doi.org/10.3389/fimmu.2021.785736 (grumach2021angioedemawithoutwheals pages 2-3); López-Lera et al., 2019, Clin Exp Immunol, doi:10.1111/cei.13361, https://doi.org/10.1111/cei.13361 (lopezlera2019serumcomplexesbetween pages 1-2) | Low C1q helps distinguish AAE-C1-INH from HAE-C1-INH, though exceptions exist. |
| Associated conditions — Czech cohort | Underlying disease in 13/14 (93%): lymphoid malignancy 9/14 (64%), MGUS 3/14 (21%), autoimmune disease 1/14 (7%), no underlying disease 1/14 (7%). (sobotkova2021acquiredangioedemawith pages 1-2) | Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 1-2) | Supports strong need to investigate lymphoproliferative and autoimmune disorders. |
| Associated conditions — Bork cohort | In 44-patient cohort: MGUS 47.7%, non-Hodgkin lymphoma 27.3%, anti-C1-INH autoantibodies alone 11.4%, other conditions 4.5%, no associated disorder 9.1%. AAE led to lymphoma detection in 75% of patients with malignancy. (bork2019angioedemadueto pages 1-2) | Bork et al., 2019, Orphanet J Rare Dis, doi:10.1186/s13023-019-1043-3, https://doi.org/10.1186/s13023-019-1043-3 (bork2019angioedemadueto pages 1-2) | One of the most quantitative cohort summaries for associated disorders. |
| Prevalence / occurrence estimates | Ultra-rare. Reported prevalence estimates: ~1:760,000 in Czech Republic; literature estimate 1:100,000 to 1:500,000; one review cites ~0.15 per 100,000. AAE-C1-INH represented ~8% of angioedema with C1-INH deficiency in the Czech study. (sobotkova2021acquiredangioedemawith pages 1-2, johnson2023aretrospectiveanalysis pages 1-2, lopezlera2019serumcomplexesbetween pages 1-2) | Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 1-2); Johnson et al., 2023, Clin Rev Allergy Immunol, doi:10.1007/s12016-023-08972-2, https://doi.org/10.1007/s12016-023-08972-2 (johnson2023aretrospectiveanalysis pages 1-2); López-Lera et al., 2019, Clin Exp Immunol, doi:10.1111/cei.13361, https://doi.org/10.1111/cei.13361 (lopezlera2019serumcomplexesbetween pages 1-2) | Incidence estimates are sparse; prevalence usually inferred from national or referral-center cohorts. |
| Phenotype frequencies — Czech cohort | Facial edema 14/14 (100%); upper airway involvement 12/14 (85.7%); abdominal attacks 7/14 (50%); peripheral angioedema 6/14 (42.8%). (sobotkova2021acquiredangioedemawith pages 4-5) | Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 4-5) | Facial and airway attacks were especially prominent in this cohort. |
| Antibody / immune-complex detection | European cohort (n=20): free anti-C1INH antibodies detected in 9/20 (45%); C1INH–anti-C1INH immune complexes detected in 18/20 (90%); 9/20 were negative for free antibodies but positive for complexes at first measurement. Hungarian cohort (n=19): 79% had an underlying disease; 11/19 had detectable anti-C1-INH antibodies at least once. (lopezlera2019serumcomplexesbetween pages 1-2, polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2) | López-Lera et al., 2019, Clin Exp Immunol, doi:10.1111/cei.13361, https://doi.org/10.1111/cei.13361 (lopezlera2019serumcomplexesbetween pages 1-2); Polai et al., 2023, Orphanet J Rare Dis, doi:10.1186/s13023-023-02625-5, https://doi.org/10.1186/s13023-023-02625-5 (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2) | Measuring complexes alongside free antibody may improve diagnostic yield and monitoring. |
| Acute treatment effectiveness | Plasma-derived C1-INH (pdC1-INH) was effective in 3553/3636 attacks (97.7%) and shortened attacks by mean 54.4 ± 32.8 hours; effectiveness in anti-C1-INH autoantibody-positive patients was 1246/1329 attacks (93.8%). Czech cohort: icatibant (n=8), pdC1-INH/Berinert (n=4), and recombinant C1-INH (n=4) were all reported effective in all treated cases. (bork2019angioedemadueto pages 7-8, sobotkova2021acquiredangioedemawith pages 4-5, bork2019angioedemadueto pages 1-2) | Bork et al., 2019, Orphanet J Rare Dis, doi:10.1186/s13023-019-1043-3, https://doi.org/10.1186/s13023-019-1043-3 (bork2019angioedemadueto pages 7-8, bork2019angioedemadueto pages 1-2); Sobotkova et al., 2021, Int Arch Allergy Immunol, doi:10.1159/000512933, https://doi.org/10.1159/000512933 (sobotkova2021acquiredangioedemawith pages 4-5) | No therapies are specifically approved for AAE-C1-INH in many regions; use is often extrapolated from HAE. |
| Prophylaxis / QoL real-world data | Berotralstat real-world series included 3 AAE-C1-INH patients. After 6 months, median attacks/month fell from 2.3 to 1.0; no aerodigestive attacks were noted; mean AE-QoL improved by 13.7 points; AECT increased by 4.2 points. (johnson2023aretrospectiveanalysis pages 1-2) | Johnson et al., 2023, Clin Rev Allergy Immunol, doi:10.1007/s12016-023-08972-2, https://doi.org/10.1007/s12016-023-08972-2 (johnson2023aretrospectiveanalysis pages 1-2) | Small sample, off-label use, but among the most relevant recent 2023 real-world AAE prophylaxis/QoL data. |
Table: This table summarizes the main clinical, laboratory, epidemiologic, and treatment facts for acquired angioedema due to C1-inhibitor deficiency using only the gathered evidence. It highlights quantitative cohort findings and recent real-world treatment data that are particularly useful for a disease knowledge base.
Several retrieved sources did not include PMIDs in the text snippets available to the tool; therefore, this report provides DOIs/URLs and publication dates from the retrieved metadata, and does not fabricate PMIDs.
References
(OpenTargets Search: Acquired angioedema): Open Targets Query (Acquired angioedema, 11 results). Buniello, A. et al. (2025). Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery. Nucleic Acids Research.
(bork2019angioedemadueto pages 1-2): Konrad Bork, Petra Staubach-Renz, and Jochen Hardt. Angioedema due to acquired c1-inhibitor deficiency: spectrum and treatment with c1-inhibitor concentrate. Orphanet Journal of Rare Diseases, Mar 2019. URL: https://doi.org/10.1186/s13023-019-1043-3, doi:10.1186/s13023-019-1043-3. This article has 68 citations and is from a peer-reviewed journal.
(sobotkova2021acquiredangioedemawith pages 1-2): Marta Sobotkova, Radana Zachova, Roman Hakl, Pavel Kuklinek, Pavlina Kralickova, Irena Krcmova, Jana Hanzlikova, Martina Vachova, and Jirina Bartunkova. Acquired angioedema with c1 inhibitor deficiency: occurrence, clinical features, and management: a nationwide retrospective study in the czech republic patients. International Archives of Allergy and Immunology, 182:642-649, Jan 2021. URL: https://doi.org/10.1159/000512933, doi:10.1159/000512933. This article has 33 citations and is from a peer-reviewed journal.
(grumach2021angioedemawithoutwheals pages 2-3): Anete S. Grumach, Camila L. Veronez, Dorottya Csuka, and Henriette Farkas. Angioedema without wheals: challenges in laboratorial diagnosis. Frontiers in Immunology, Dec 2021. URL: https://doi.org/10.3389/fimmu.2021.785736, doi:10.3389/fimmu.2021.785736. This article has 26 citations and is from a peer-reviewed journal.
(johnson2023aretrospectiveanalysis pages 1-2): Felix Johnson, Anna Stenzl, Benedikt Hofauer, Helen Heppt, Eva-Vanessa Ebert, Barbara Wollenberg, Robin Lochbaum, Janina Hahn, Jens Greve, and Susanne Trainotti. A 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 & Immunology, 65:354-364, Nov 2023. URL: https://doi.org/10.1007/s12016-023-08972-2, doi:10.1007/s12016-023-08972-2. This article has 14 citations and is from a peer-reviewed journal.
(polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2): Zsofia Polai, Erika Kajdacsi, Laszlo Cervenak, Zsuzsanna Balla, Szabolcs Benedek, Lilian Varga, and Henriette Farkas. C1-inhibitor/c1-inhibitor antibody complexes in acquired angioedema due to c1-inhibitor deficiency. Orphanet Journal of Rare Diseases, Feb 2023. URL: https://doi.org/10.1186/s13023-023-02625-5, doi:10.1186/s13023-023-02625-5. This article has 6 citations and is from a peer-reviewed journal.
(NCT07266805 chunk 1): Study of Oral Deucrictibant XR Tablet for Prophylaxis and Deucrictibant IR Capsule for On-Demand Treatment of Angioedema Attacks in Adults With Acquired Angioedema Due to C1 Inhibitor Deficiency. Pharvaris Netherlands B.V.. 2025. ClinicalTrials.gov Identifier: NCT07266805
(sobotkova2021acquiredangioedemawith pages 4-5): Marta Sobotkova, Radana Zachova, Roman Hakl, Pavel Kuklinek, Pavlina Kralickova, Irena Krcmova, Jana Hanzlikova, Martina Vachova, and Jirina Bartunkova. Acquired angioedema with c1 inhibitor deficiency: occurrence, clinical features, and management: a nationwide retrospective study in the czech republic patients. International Archives of Allergy and Immunology, 182:642-649, Jan 2021. URL: https://doi.org/10.1159/000512933, doi:10.1159/000512933. This article has 33 citations and is from a peer-reviewed journal.
(caballero2022medicalalgorithmmanagement pages 2-2): Teresa Caballero, Rosario Cabañas, and María Pedrosa. Medical algorithm: management of c1 inhibitor hereditary angioedema. Allergy, 77:1060-1063, Oct 2022. URL: https://doi.org/10.1111/all.15115, doi:10.1111/all.15115. This article has 4 citations and is from a highest quality peer-reviewed journal.
(lopezlera2019serumcomplexesbetween pages 1-2): A. López-Lera, S. Garrido, P. Nozal, Lillemor Skatum, A. Bygum, T. Caballero, and M. López Trascasa. Serum complexes between c1inh and c1inh autoantibodies for the diagnosis of acquired angioedema. Clinical & Experimental Immunology, 198:341-350, Dec 2019. URL: https://doi.org/10.1111/cei.13361, doi:10.1111/cei.13361. This article has 12 citations and is from a peer-reviewed journal.
(falco2025orofacialangioedemaan pages 3-5): Domenico De Falco, Diego Misceo, Giuseppe Carretta, Gioele Gioco, Carlo Lajolo, and Massimo Petruzzi. Oro-facial angioedema: an overview. Immuno, 5:61, Dec 2025. URL: https://doi.org/10.3390/immuno5040061, doi:10.3390/immuno5040061. This article has 2 citations.
(NCT06818474 chunk 1): Jonathan A. Bernstein, MD. Lanadelumab in Long-term Prophylaxis of Acquired Angioedema. Bernstein Clinical Research Center. 2024. ClinicalTrials.gov Identifier: NCT06818474
(bork2019angioedemadueto pages 7-8): Konrad Bork, Petra Staubach-Renz, and Jochen Hardt. Angioedema due to acquired c1-inhibitor deficiency: spectrum and treatment with c1-inhibitor concentrate. Orphanet Journal of Rare Diseases, Mar 2019. URL: https://doi.org/10.1186/s13023-019-1043-3, doi:10.1186/s13023-019-1043-3. This article has 68 citations and is from a peer-reviewed journal.