Acquired Angioedema

1. Disease Information

1.1 Concise overview (current understanding)

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)

1.2 Common synonyms / alternative names

• Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) (sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto pages 1-2)
• Acquired C1-inhibitor deficiency (johnson2023aretrospectiveanalysis pages 1-2)
• C1-INH-AAE (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)

1.3 Key identifiers (availability in retrieved sources)

• MONDO: MONDO:0019624 (OpenTargets mapping) (OpenTargets Search: Acquired angioedema)
• Other identifiers requested (ICD-10/ICD-11, Orphanet, MeSH, OMIM): Not retrievable from the currently collected tool evidence; this report therefore does not assert specific ICD/Orphanet/MeSH/OMIM codes.

1.4 Evidence source types

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)

2. Etiology

2.1 Primary causes (mechanistic)

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)

2.2 Risk factors / associated conditions (high-confidence)

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)

2.3 Triggers and precipitating factors

Triggers reported include mechanical trauma, emotional stress, and ACE inhibitors (as potential triggers/complicating exposures in bradykinin-mediated disease contexts). (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)

2.4 Protective factors and gene–environment interactions

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)

3. Phenotypes

3.1 Clinical phenotype spectrum and frequencies

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)

3.2 Phenotype characteristics

• Age of onset: Typically adult-onset, often >40 years; cohort median near 60 years. (sobotkova2021acquiredangioedemawith pages 1-2, sobotkova2021acquiredangioedemawith pages 4-5)
• Course pattern: Episodic attacks.
• Severity: Potentially life-threatening due to airway involvement. (bork2019angioedemadueto pages 1-2)

3.3 Quality-of-life (QoL) impact

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)

3.4 Suggested HPO terms (non-exhaustive)

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)

4. Genetic / Molecular Information

4.1 Causal genes and variants

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)

4.2 Autoantibodies and immune complexes (key molecular entities)

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)

4.3 Suggested CHEBI entities (therapeutic-relevant)

• Bradykinin: CHEBI:15883 (central mediator; evidence of bradykinin-mediated mechanism from reviews/algorithms) (bork2019angioedemadueto pages 1-2, grumach2021angioedemawithoutwheals pages 2-3)

5. Environmental Information

5.1 Environmental/lifestyle factors

No population-level environmental or lifestyle risk factor evidence specific to AAE-C1-INH was captured in the retrieved documents.

5.2 Drug-related triggers

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)

6. Mechanism / Pathophysiology

6.1 Causal chain (trigger → mediator → phenotype)

1. Underlying B-cell lymphoproliferation/MGUS and/or autoantibodies leads to consumption or inactivation of C1-INH. (sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto pages 1-2, johnson2023aretrospectiveanalysis pages 1-2)
2. Reduced functional C1-INH permits dysregulated activation of complement/contact systems, increasing downstream generation of bradykinin. (grumach2021angioedemawithoutwheals pages 2-3, bork2019angioedemadueto pages 1-2)
3. Bradykinin increases vascular permeability, producing episodic submucosal/subcutaneous edema (angioedema), including potentially fatal laryngeal edema. (bork2019angioedemadueto pages 1-2, grumach2021angioedemawithoutwheals pages 2-3)

6.2 Upstream vs downstream

• Upstream: Underlying disease (lymphoproliferative, MGUS, autoimmune), anti–C1-INH antibodies/complexes, complement consumption (low C1q suggests classical pathway involvement). (sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto pages 1-2, lopezlera2019serumcomplexesbetween pages 1-2)
• Downstream: Bradykinin-mediated permeability and tissue swelling; failure to respond to histamine-directed therapies is a practical downstream clinical discriminator in reviews/algorithms. (falco2025orofacialangioedemaan pages 3-5, caballero2022medicalalgorithmmanagement pages 2-2)

6.3 Suggested GO Biological Process terms (examples)

• Complement activation, classical pathway: GO:0006958 (supported by complement consumption patterns, low C1q) (sobotkova2021acquiredangioedemawith pages 4-5, grumach2021angioedemawithoutwheals pages 2-3)
• Regulation of blood vessel permeability: GO:0008217 (bradykinin-mediated edema phenotype) (bork2019angioedemadueto pages 1-2)

6.4 Suggested Cell Ontology (CL) terms (best-effort)

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)

7. Anatomical Structures Affected

7.1 Primary anatomical sites and systems (with UBERON suggestions)

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)

8. Temporal Development

8.1 Onset and course

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)

8.2 Remission patterns

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)

9. Inheritance and Population

9.1 Inheritance pattern

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)

9.2 Epidemiology (statistics)

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)

10. Diagnostics

10.1 Core diagnostic laboratory tests (complement/C1-INH panel)

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)

10.2 Antibody/complex testing as a diagnostic enhancer (recent development)

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)

10.3 Differential diagnosis (high-level)

• Hereditary angioedema due to C1-INH deficiency (HAE types I/II): similar clinical phenotype but typically earlier onset and normal C1q; SERPING1 variants may be present. (grumach2021angioedemawithoutwheals pages 2-3, caballero2022medicalalgorithmmanagement pages 2-2)
• Histaminergic angioedema: tends to respond to antihistamines/corticosteroids/epinephrine; complement panel typically normal. (falco2025orofacialangioedemaan pages 3-5, caballero2022medicalalgorithmmanagement pages 2-2)
• ACE inhibitor–associated angioedema: bradykinin-mediated but lacks the characteristic complement abnormalities; diagnosis is clinical/exclusion. (grumach2021angioedemawithoutwheals pages 2-3)

10.4 Suggested LOINC-style test names (best-effort)

(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)

11. Outcome / Prognosis

11.1 Morbidity and mortality considerations

AAE-C1-INH can be life-threatening due to laryngeal edema/asphyxiation risk. (bork2019angioedemadueto pages 1-2)

11.2 Prognostic factors (inferred from cohort observations)

• Presence and treatability of an underlying lymphoproliferative disorder is clinically important; treating lymphoma was associated with reduced angioedema activity and complement normalization in a Czech cohort summary. (sobotkova2021acquiredangioedemawith pages 1-2)

Robust survival rates, mortality rates, or long-term disability estimates were not present in the retrieved tool evidence.

12. Treatment

12.1 Current applications and real-world implementations

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)

On-demand (acute attack) treatments

• Plasma-derived C1-INH concentrate (pdC1-INH): In a 44-patient referral cohort, pdC1-INH was reported effective in 3553/3636 attacks (97.7%) and shortened attack duration by 54.4 ± 32.8 hours on average; effectiveness remained high even in anti–C1-INH antibody-positive patients (93.8% effectiveness). (bork2019angioedemadueto pages 1-2)
• Icatibant (bradykinin B2 receptor antagonist): In Czech cohort, icatibant was used (n=8) and reported efficient in all treated cases. (sobotkova2021acquiredangioedemawith pages 4-5)
• Recombinant C1-INH: used acutely in Czech cohort (n=4), efficient in all treated cases. (sobotkova2021acquiredangioedemawith pages 4-5)

Long-term prophylaxis and disease control (recent evidence; 2023 focus)

• Berotralstat (oral plasma kallikrein inhibitor): A 2023 real-world retrospective series included 3 AAE-C1-INH patients; after 6 months, median attacks/month decreased from 2.3 to 1.0, with AE-QoL improvement of 13.7 points and AECT increase of 4.2 points. (johnson2023aretrospectiveanalysis pages 1-2)
• Tranexamic acid (TA): In Czech cohort, long-term prophylaxis was started in 9/14; TA was used in 5, and was effective as a single agent in 2/5. (sobotkova2021acquiredangioedemawith pages 4-5)

Treating underlying disease (mechanism-directed upstream therapy)

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)

12.2 Experimental / clinical trials (recent developments; 2024–2026 registry data)

• Lanadelumab in long-term prophylaxis of acquired angioedema (NCT06818474): Phase 4, open-label, single-group study; inclusion specifies recurrent AAE without urticaria plus labs consistent with acquired C1-INH deficiency (decreased C1INH functional/quantitative, decreased C4, decreased C1q, no family history, anti-C1INH Ab and/or paraproteinemia). Start date 2024-06-01; first posted 2025-02-10. URL: https://clinicaltrials.gov/ct2/show/NCT06818474 (NCT06818474 chunk 1)
• Deucrictibant (oral bradykinin B2 receptor antagonist) for AAE-C1INH (NCT07266805, “CREAATE”): Phase 3 randomized placebo-controlled multi-part study testing XR prophylaxis and IR on-demand treatment; start date 2025-10-16; first posted 2025-12-05; last update 2026-05-07. URL: https://clinicaltrials.gov/study/NCT07266805 (NCT07266805 chunk 1)

12.3 Suggested MAXO terms (examples; best-effort)

• C1 esterase inhibitor replacement therapy (pdC1-INH/rhC1-INH) (bork2019angioedemadueto pages 1-2, sobotkova2021acquiredangioedemawith pages 4-5)
• Bradykinin receptor antagonist therapy (icatibant; and oral B2 antagonist under trial) (sobotkova2021acquiredangioedemawith pages 4-5, NCT07266805 chunk 1)
• Kallikrein inhibitor therapy (berotralstat; lanadelumab) (johnson2023aretrospectiveanalysis pages 1-2, NCT06818474 chunk 1)
• Antifibrinolytic therapy (tranexamic acid) (sobotkova2021acquiredangioedemawith pages 4-5)
• Treatment of underlying lymphoproliferative disease (sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto pages 1-2)

13. Prevention

13.1 Prevention levels

• Primary prevention: Not well-defined because AAE-C1-INH is typically secondary to underlying conditions rather than preventable exposures.
• Secondary/tertiary prevention: Key strategy is early recognition of bradykinin-mediated angioedema, confirmation with complement testing, and preventing airway compromise through rapid access to effective on-demand therapy; additionally, identifying/treating underlying lymphoproliferative disease can reduce recurrence. (sobotkova2021acquiredangioedemawith pages 1-2, bork2019angioedemadueto 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)

14. Other Species / Natural Disease

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.

15. Model Organisms

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.

Key quantitative facts table (for knowledge base ingestion)

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.

Expert opinion and analysis (synthesis of authoritative sources)

1. Diagnostic emphasis: Multiple authoritative reviews/algorithms converge on the idea that AAE-C1-INH is best recognized by combining clinical context (late onset, no family history) with a complement/C1-INH laboratory panel including C1q and optional anti–C1-INH testing; however, these markers can show exceptions, so repeated testing and comprehensive evaluation for underlying B-cell disease are often required. (grumach2021angioedemawithoutwheals pages 2-3, caballero2022medicalalgorithmmanagement pages 2-2, sobotkova2021acquiredangioedemawith pages 4-5)
2. Recent methodological refinement (2023): Measuring C1-INH/anti–C1-INH complexes (CAC) in parallel with free antibodies is a notable 2023 development aimed at improving detection and potentially monitoring underlying disease evolution. (polai2023c1inhibitorc1inhibitorantibodycomplexes pages 1-2)
3. Treatment reality: Despite the absence of universally approved AAE-specific therapies, real-world cohort data support high effectiveness of pdC1-INH for acute attacks, and emerging prophylaxis approaches (e.g., oral kallikrein inhibition with berotralstat) show promising reductions in attack rate and improved patient-reported outcomes in small AAE subsets. (bork2019angioedemadueto pages 1-2, johnson2023aretrospectiveanalysis pages 1-2)

Notes on citation requirements (PMID availability)

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

1. (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.

2. (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.

3. (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.

4. (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.

5. (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.

6. (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.

7. (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

8. (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.

9. (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.

10. (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.

11. (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.

12. (NCT06818474 chunk 1): Jonathan A. Bernstein, MD. Lanadelumab in Long-term Prophylaxis of Acquired Angioedema. Bernstein Clinical Research Center. 2024. ClinicalTrials.gov Identifier: NCT06818474

13. (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.