Autoimmune angioedema

Overview

Autoimmune angioedema is an immune-mediated condition characterized by recurrent episodes of deep swelling in the skin, mucous membranes, or internal organs. Unlike hereditary angioedema, which is caused by genetic mutations, autoimmune angioedema happens when the immune system mistakenly attacks proteins that help control inflammation. This leads to excessive swelling because the body is unable to properly regulate fluid movement in tissues.

Common Symptoms

Swelling of the face, lips, tongue, throat, or extremities; abdominal pain; difficulty breathing due to airway swelling; and episodes that may occur spontaneously or be triggered by stress, infections, or medications.

Coexisting Diseases and Conditions

Systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome, chronic urticaria, immune thrombocytopenia, lymphoproliferative disorders.

Risk Factors and Prevalence

Autoimmune angioedema is rare and often misdiagnosed. It primarily affects individuals with underlying autoimmune diseases. The presence of C1-inhibitor deficiency or autoantibodies against C1-esterase inhibitor is a key diagnostic marker. Women may be more frequently affected, as with many autoimmune conditions.

Recent Research

Sources

Sources
1. Kaplan, A. P., & Greaves, M. W. (2005). Angioedema. Journal of the American Academy of Dermatology, 53(3), 373–392. https://doi.org/10.1016/j.jaad.2004.09.032
2. Cicardi, M., Aberer, W., Banerji, A., Bas, M., Bernstein, J. A., Bork, K., Caballero, T., Farkas, H., Grumach, A., Kaplan, A. P., Riedl, M. A., Triggiani, M., Zanichelli, A., Zuraw, B., & HAWK under the patronage of EAACI (European Academy of Allergy and Clinical Immunology) (2014). Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Allergy, 69(5), 602–616. https://doi.org/10.1111/all.12380
3. Longhurst, H. J., & Bork, K. (2019). Hereditary angioedema: an update on causes, manifestations and treatment. British journal of hospital medicine (London, England : 2005), 80(7), 391–398. https://doi.org/10.12968/hmed.2019.80.7.391
4. Jindal, A. K., Bishnoi, A., & Dogra, S. (2021). Hereditary Angioedema: Diagnostic Algorithm and Current Treatment Concepts. Indian dermatology online journal, 12(6), 796–804. https://doi.org/10.4103/idoj.idoj_398_21