Efficacy of Lanadelumab in acquired angioedema with C1-inhibitor deficiency

2021 ◽  
Author(s):  
Aude Belbézier ◽  
Isabelle Boccon-Gibod ◽  
Laurence Bouillet
Keyword(s):  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema

Acquired angioedema with C1-inhibitor deficiency: similarities and differences with hereditary angioedema

Alergologia ◽  
2019 ◽  
Vol 1 (3) ◽  
pp. 27
Author(s):  
Noémi-Anna Bara ◽  
Valentin Nădăşan
Keyword(s):  
Hereditary Angioedema ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema ◽  
Similarities And Differences

scholarly journals Acquired Angioedema with C1 Inhibitor Deficiency Associated with Anticardiolipin Antibodies

2011 ◽  
Vol 24 (4) ◽  
pp. 1115-1118 ◽  
Author(s):  
E. Di Leo ◽  
E. Nettis ◽  
V. Montinaro ◽  
G. Calogiuri ◽  
P. Delle Donne ◽  
...  
Keyword(s):  
Anticardiolipin Antibodies ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema

scholarly journals Epidemiology and Management of Acquired Angioedema Due to C1 Inhibitor Deficiency

2021 ◽  
pp. 107-113
Author(s):  
Mohammed Olaythah Alraddadi ◽  
Yousef Hussain J. Alharthi ◽  
Rayyan Fahad H. Altemani ◽  
Wejdan Mohammed S. Alshehri ◽  
Amal Nafea J. Alharbi ◽  
...  
Keyword(s):  
Receptor Antagonist ◽  
Published Data ◽  
Efficacy And Safety ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema ◽  
Bradykinin B2 Receptor ◽  
On Demand

AAE-C1-INH (acquired angioedema owing to C1-inhibitor (C1-INH) deficiency) is a dangerous illness that can lead to asphyxiation due to laryngeal edoema. Only around 1% to 2% of angioedema cases are classified as HAE or AAE, with HAE being 10 times more prevalent than AAE. The sole clinical distinction between HAE and AAE is the age at which symptoms appea, AAE-C1-INH is usually diagnosed after 40 years of age. There is no licensed therapy for AAE-C1-INH at this time. AAE-C1-INH attacks are treated with HAE-C1-INH medicines such plasma-derived C1-INH concentrate (pdC1-INH) and the bradykinin B2 receptor antagonist, icatibant. These on-demand medications are thought to be most helpful when provided early in the attack. However, there is a scarcity of published data on the efficacy and safety of AAE-C1-INH therapies.

scholarly journals Malignancy and immune disorders in patients with hereditary angioedema

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Peter Stepaniuk ◽  
Amin Kanani
Keyword(s):  
Hereditary Angioedema ◽  
Cervical Dysplasia ◽  
Case Series ◽  
Immune Disorders ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Immune Disorder ◽  
Acquired Angioedema ◽  
Increased Risk ◽  
High Index Of Suspicion

Abstract Background Hereditary angioedema (HAE) is an inherited condition manifesting as recurrent angioedema episodes which is caused by deficiency or dysfunction of C1 inhibitor. Although complement dysregulation has historically been shown to be associated with various malignancy and immune disorders, it is currently not known if HAE patients are at an increased risk of developing malignancy or autoimmune conditions. Case presentation We reviewed the charts of 49 HAE patients and identified 6 patients who had a co-existing malignancy diagnosis (two with breast cancer, one with melanoma, one with pancreatic cancer, one with renal cancer and one with cervical dysplasia) and 6 patients who had a diagnosis of a co-existing immune disorder (two with rheumatoid arthritis, two with ulcerative colitis, one with chronic urticaria with hypothyroidism and one with Sjogren’s syndrome). Nearly all malignancy cases occurred in older HAE patients (> 50 years) and malignancy was diagnosed before HAE in 3 of the patients. Conclusions Our case series identified multiple hereditary angioedema (HAE) patients with co-existing malignancy and immune disorders. Based on these findings, we would advocate that physicians managing HAE patients should maintain a high index of suspicion for these conditions and that in patients with angioedema, C1 inhibitor deficiency and malignancy, a diagnosis of HAE should still be considered in addition to acquired angioedema (AAE).

scholarly journals Acquired angioedema due to C1-inhibitor deficiency: a survey of 101 Italian patients

2017 ◽  
Vol 139 (2) ◽  
pp. AB232
Author(s):  
Mauro Cancian ◽  
Andrea Zanichelli ◽  
Francesco Arcoleo ◽  
Maria Bova ◽  
Riccardo Senter ◽  
...  
Keyword(s):  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema

Rituximab-induced Elimination of Acquired Angioedema Due to C1-Inhibitor Deficiency

2006 ◽  
Vol 119 (8) ◽  
pp. e3-e5 ◽  
Author(s):  
Marcel Levi ◽  
C. Erik Hack ◽  
Marinus H. van Oers
Keyword(s):  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema

Acquired Angioedema due to C1 Inhibitor Deficiency Preceding Splenic Marginal Zone Lymphoma: Further Insights from Clinical Practice

2020 ◽  
Vol 181 (12) ◽  
pp. 941-946
Author(s):  
Mariana Paes Leme Ferriani ◽  
Orlando Trevisan-Neto ◽  
Julia S. Costa ◽  
Janaina M.L. Melo ◽  
Adriana S. Moreno ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema ◽  
Risk Of Death ◽  
Increased Risk

<b><i>Background:</i></b> Acquired angioedema due to C1 inhibitor deficiency (AAE-C1-INH) is a very rare disease. In clinical practice, it may be difficult to differentiate AAE-C1-INH from hereditary angioedema due to C1-INH deficiency (HAE-C1-INH). In both conditions, patients are at an increased risk of death from asphyxiation due to upper airway obstruction. The association of AAE-C1-INH with lymphoproliferative and autoimmune diseases, and with presence of anti-C1-INH antibodies has been well documented, and treatment of the underlying condition may result in complete remission of angioedema. <b><i>Objectives:</i></b> To discuss the clinical evaluation, diagnosis, and treatment outcomes of AAE-C1-INH in the context of the care of 2 patients with recurrent isolated angioedema. <b><i>Methods:</i></b> Two patients were followed up prospectively at our clinic. Measurements of C3, C4, C1-INH, and C1q levels were carried out by nephelometry, and the functional activity of C1-INH was determined by a chromogenic assay. Hematological investigation included morphological and immunophenotyping analysis of peripheral blood, bone marrow, and spleen histopathology. Sequencing of the 8 exons and adjacent intronic regions of the <i>SERPING1</i> gene was performed using the Sanger method. <b><i>Results:</i></b> Two patients were diagnosed with AAE-C1-INH associated with splenic marginal zone lymphoma during follow-up. <b><i>Conclusions:</i></b> Close follow-up, including detailed clinical history, physical examination, and laboratory tests, of our patients with AAE-C1-INH was essential for the early diagnosis and successful treatment of the lymphoproliferative disease, leading to the resolution of the angioedema attacks.

Long‐term effects upon rituximab treatment of acquired angioedema due to C1‐inhibitor deficiency

Allergy ◽  
2018 ◽  
Vol 74 (4) ◽  
pp. 834-840 ◽  
Author(s):  
Marcel Levi ◽  
Danny Cohn ◽  
Sacha Zeerleder ◽  
Magdalena Dziadzio ◽  
Hilary Longhurst
Keyword(s):  
C1 Inhibitor ◽  
Long Term Effects ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema
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