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

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.

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Malignancy and immune disorders in patients with hereditary angioedema

Allergy Asthma & Clinical Immunology ◽

10.1186/s13223-021-00621-7 ◽

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).

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

Journal of Allergy and Clinical Immunology ◽

10.1016/j.jaci.2016.12.747 ◽

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

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

The American Journal of Medicine ◽

10.1016/j.amjmed.2005.09.018 ◽

2006 ◽

Vol 119 (8) ◽

pp. e3-e5 ◽

Cited By ~ 40

Author(s):

Marcel Levi ◽

C. Erik Hack ◽

Marinus H. van Oers

Keyword(s):

C1 Inhibitor ◽

C1 Inhibitor Deficiency ◽

Acquired Angioedema

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

International Archives of Allergy and Immunology ◽

10.1159/000509805 ◽

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

Background: 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. Objectives: 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. Methods: 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 SERPING1 gene was performed using the Sanger method. Results: Two patients were diagnosed with AAE-C1-INH associated with splenic marginal zone lymphoma during follow-up. Conclusions: 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.

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