Autoimmune urticaria
Overview
Autoimmune urticaria (also called autoimmune chronic spontaneous urticaria [aiCSU] and formerly known as chronic autoimmune urticaria) is a form of chronic spontaneous urticaria in which the immune system mistakenly activates mast cells, leading to recurrent hives (urticaria), swelling beneath the skin (angioedema), or both.
Current research recognizes two major autoimmune endotypes. Type I (autoallergic) is driven by IgE autoantibodies against self-antigens, whereas type IIb autoimmune urticaria is driven by IgG autoantibodies directed against IgE or its high-affinity receptor (FcεRI).
Common Symptoms
Recurrent red or skin-colored hives (welts) that appear and fade repeatedly, intense itching, angioedema (swelling beneath the skin), painful swelling of the eyelids, lips, face, hands, or throat, and symptoms that persist or recur for more than six weeks.
Coexisting Diseases and Conditions
Autoimmune thyroid disease (especially Hashimoto’s thyroiditis), vitiligo, rheumatoid arthritis, Sjögren disease, celiac disease, type 1 diabetes, systemic lupus erythematosus (SLE), and metabolic syndrome.
Risk Factors and Prevalence
Autoimmune urticaria can occur at any age but is most common in adults between 30 and 50 years of age. Women are affected significantly more often than men, with studies reporting a female-to-male ratio of approximately 2:1 to 4:1. Chronic spontaneous urticaria affects approximately 1% of the general population worldwide, and research suggests that more than half of cases involve autoimmune mechanisms. People with autoimmune thyroid disease and other autoimmune diseases may have an increased risk of developing autoimmune urticaria.
Recent Research
- Autoimmune chronic spontaneous urticaria (2022)
- Prevalence of Autoimmune and Autoinflammatory Diseases in Chronic Urticaria: Pathogenetic, Diagnostic and Therapeutic Implications (2023)
- How Infection and Vaccination Are Linked to Acute and Chronic Urticaria: A Special Focus on COVID-19 (2023)
- miRNAs’ Cross-Involvement in Skin Allergies: A New Horizon for the Pathogenesis, Diagnosis and Therapy of Atopic Dermatitis, Allergic Contact Dermatitis and Chronic Spontaneous Urticaria (2023)
- Correlation between regulatory T cells and autologous serum skin test among chronic spontaneous urticaria patients (2022)
Sources
- Sources
Kolkhir, P., Bonnekoh, H., Metz, M., & Maurer, M. (2024). Chronic Spontaneous Urticaria: A Review. JAMA, 332(17), 1464–1477. https://doi.org/10.1001/jama.2024.15568
Cassano, N., Colombo, D., Bellia, G., Zagni, E., & Vena, G. A. (2016). Gender-related differences in chronic urticaria. Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia, 151(5), 544–552.
Kolkhir, P., Muñoz, M., Asero, R., Ferrer, M., Kocatürk, E., Metz, M., Xiang, Y. K., & Maurer, M. (2022). Autoimmune chronic spontaneous urticaria. The Journal of allergy and clinical immunology, 149(6), 1819–1831. https://doi.org/10.1016/j.jaci.2022.04.010
Hamel-Teillac, D. (2003). [Chronic urticaria in children. Annales de dermatologie et de venereologie, 130 Spec No 1, 1S69-72.
Lee, S. J., Ha, E. K., Jee, H. M., Lee, K. S., Lee, S. W., Kim, M. A., Kim, D. H., Jung, Y.-H., Sheen, Y. H., Sung, M. S., & Han, M. Y. (2017). Prevalence and Risk Factors of Urticaria With a Focus on Chronic Urticaria in Children. Allergy, Asthma & Immunology Research, 9(3), 212–219. https://doi.org/10.4168/aair.2017.9.3.212
Larenas-Linnemann D. (2023). Biomarkers of Autoimmune Chronic Spontaneous Urticaria. Current allergy and asthma reports, 23(12), 655–664. https://doi.org/10.1007/s11882-023-01117-7
Bracken, S. J., Abraham, S., & MacLeod, A. S. (2019). Autoimmune Theories of Chronic Spontaneous Urticaria. Frontiers in immunology, 10, 627. https://doi.org/10.3389/fimmu.2019.00627
Oliver, E. T., & Saini, S. S. (2024). Chronic Spontaneous Urticaria: Etiology and Pathogenesis. Immunology and allergy clinics of North America, 44(3), 421–438. https://doi.org/10.1016/j.iac.2024.03.002
Muñoz, M., Kiefer, L. A., Pereira, M. P., Bizjak, M., & Maurer, M. (2024). New insights into chronic inducible urticaria. Current allergy and asthma reports, 24(8), 457–469. https://doi.org/10.1007/s11882-024-01160-y