Comorbidity and pathogenic links of chronic spontaneous urticaria and systemic lupus erythematosus – a systematic review

Abstract

Chronic spontaneous urticaria (CSU) is a common mast cell-driven disease characterized by the development of wheals (hives), angioedema (AE), or both for > 6 weeks. It is thought that autoimmunity is a common cause of CSU, which is often associated with autoimmune thyroiditis, whereas the link to other autoimmune disorders such as systemic lupus erythematosus (SLE) has not been carefully explored. Here, we systematically reviewed the existing literature for information on the prevalence of CSU in SLE (and vice versa) and we examined the possible clinical and pathogenetic relationship between CSU and SLE. The prevalence of CSU and CSU-like rash in SLE was investigated by 42 independent studies and comorbidity in adult patients reportedly ranged from 0% to 21.9% and 0.4% to 27.5%, respectively (urticarial vasculitis: 0-20%). In children with SLE, CSU was reported in 0-1.2% and CSU-like rash in 4.5-12% (urticarial vasculitis: 0-2.2%). In contrast, little information is available on the prevalence of SLE in patients with CSU, and more studies are needed to determine the rate of comorbidity. Recent insights on IgG- and IgE-mediated autoreactivity suggest similarities in the pathogenesis of CSU and SLE linking inflammation and autoimmunity with the activation of the complement and coagulation system. Future studies of patients with either or both conditions could help to better define common pathomechanisms in CSU and SLE and to develop novel targeted treatment options for patients with CSU and SLE.

Impact of chronic urticaria on systemic lupus erythematosus: A nationwide population‐based study in Taiwan

“…Approximately 40% of cases of CU are characterized by production of IgG autoantibodies . Chronic spontaneous urticaria and urticarial vasculitis are the two most common underlying causes of CU rash in SLE.…”

“…Early diagnosis and appropriate treatment involving clinicians across many different specialties are crucial for preventing further manifestations and achieving beneficial long‐term outcomes for patients . Urticaria may present as the initial symptom in these patients before the more classical form of SLE is confirmed . CU‐like lesions may occur in lupus tumidus, urticarial plaque of LE, which is an uncommon LE‐specific but distinct subtype of chronic cutaneous LE…”

Comorbidities in Chronic Spontaneous Urticaria

“…These autoantibodies bind to mast cell-bound IgE molecules or surface IgE receptors to stimulate and eventually degranulate the cells and cause the urticarial symptoms [17][18][19]. It was suggested that some CSU patients have an autoimmune mechanism induced by these autoantibodies [20].…”

“…Thyroid diseases, especially Hashimoto's disease in which production of thyroid autoantibodies (antithyroid peroxidase antibodies and antithyroglobulin antibodies) and lymphocytic infiltration into the thyroid gland are seen, are the most common autoimmune diseases accompanying patients with CSU [19][20][21]. The etiology of thyroid autoimmunity is not known, but the genetic susceptibility and environmental factors are thought to initiate the process [22].…”

“…Although autoimmune thyroid disorders have been investigated in CSU, its relation with other autoimmune diseases has been investigated less [20]. But there is a fact that if there is an autoimmune disease in a patient, the second or third autoimmune disease more often appears even if the patient is under immunosuppressive treatment [21].…”