Allergy & Immunology

Chronic Spontaneous Urticaria

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Chronic Spontaneous Urticaria in Children and Adults: Presentation, Course, and Management Considerations

patient care perspectives by Thomas B. Casale, MD
Overview

Chronic spontaneous urticaria (CSU) can have a significant impact on individual quality of life. Understanding the differences in disease presentation, course, and severity between children and adults can aid in the appropriate diagnosis and management of the condition.

Expert Commentary
“CSU is more common in adults than in children. . . . In children, the duration of CSU tends to be shorter, whereas it can last for years in adults. The severity of CSU also appears to be worse in adults, who have more angioedema and a greater likelihood of having biomarkers for autoimmunity. . . .”
— Thomas B. Casale, MD

CSU is more common in adults than in children, the latter of whom more commonly present with acute urticaria. The differential diagnosis for CSU can be confusing, as there are other underlying disorders that might also present with urticaria and may be confused with CSU, such as lupus or urticarial vasculitis, and these are also more common in adults than in children.

 

In children, the duration of CSU tends to be shorter, whereas it can last for years in adults. The severity of CSU also appears to be worse in adults, who have more angioedema and a greater likelihood of having biomarkers for autoimmunity, such as autoantibodies against TPO, IgE, or the high-affinity IgE receptor, which can trigger mast cell degranulation. Issues of autoimmunity also make adults with CSU more difficult to treat than children, who often do not have these biomarkers present. Moreover, ACE inhibitors and NSAIDs can aggravate CSU, which is something to keep in mind when treating adults with the condition.

 

For both autoimmune and autoallergic CSU, treatment begins with a nonsedating, second-generation antihistamine, which can be increased in adults as needed to up to 4 times the licensed dose, if necessary. There is no evidence that sedating antihistamines are more effective than nonsedating antihistamines, and, because CSU is already associated with diminished quality of life, you do not want to add to the burden of disease by initiating a medication that is associated with sedation and impairment.

 

If patients do not respond to an antihistamine, a trial of omalizumab is the next step. However, people with autoimmune CSU respond slower to omalizumab and may not respond as well. In the United States, we do not routinely obtain biomarkers, although we may occasionally do so to counsel a patient. For example, if the patient is known to have certain autoantibodies, they can be told something like, “Omalizumab might work for you, but hang in there. It is going to take a while.”

 

Finally, it is worth noting that long-term treatment with systemic corticosteroids should be avoided in both children and adults with the disease. CSU can last for many years, and even small cumulative doses of systemic corticosteroids can have adverse consequences, including an increased risk of osteoporosis, diabetes, and cardiovascular disease, among others.

References

Chang J, Cattelan L, Ben-Shoshan M, Le M, Netchiporouk E. Management of pediatric chronic spontaneous urticaria: a review of current evidence and guidelines. J Asthma Allergy. 2021;14:187-199. doi:10.2147/JAA.S249765

 

Kolkhir P, Muñoz M, Asero R, et al. Autoimmune chronic spontaneous urticaria. J Allergy Clin Immunol. 2022;149(6):1819-1831. doi:10.1016/j.jaci.2022.04.010

 

Netchiporouk E, Sasseville D, Moreau L, Habel Y, Rahme E, Ben-Shoshan M. Evaluating comorbidities, natural history, and predictors of early resolution in a cohort of children with chronic urticaria. JAMA Dermatol. 2017;153(12):1236-1242. doi:10.1001/jamadermatol.2017.3182

 

Özçeker D, Can PK, Terzi Ö, et al. Differences between adult and pediatric chronic spontaneous urticaria from a cohort of 751 patients: clinical features, associated conditions and indicators of treatment response. Pediatr Allergy Immunol. 2023;34(2):e13925. doi:10.1111/pai.13925

 

Sánchez-Borges M, Ansotegui IJ, Baiardini I, et al. The challenges of chronic urticaria part 1: epidemiology, immunopathogenesis, comorbidities, quality of life, and management. World Allergy Organ J. 2021;14(6):100533. doi:10.1016/j.waojou.2021.100533

 

Schoepke N, Asero R, Ellrich A, et al. Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria: results of the PURIST study. Allergy. 2019;74(12):2427-2436. doi:10.1111/all.13949

Thomas B. Casale, MD

    Professor of Internal Medicine and Pediatrics
    Chief of Clinical and Translational Research
    University of South Florida Health Morsani College of Medicine
    Tampa, FL
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