Autoimmune comorbidity in chronic spontaneous urticaria: A systematic review

Background and objective Numerous autoimmune diseases (AIDs) have been linked to chronic spontaneous urticaria (CSU). Here, we provide the first extensive and comprehensive evaluation of the prevalence of AIDs in patients with CSU and vice versa. Methods A Pubmed and Google Scholar search was performed to identify studies reporting the prevalence of various AIDs in CSU and vice versa published before April 2017. Results The prevalence of individual AIDs in CSU is increased (≥ 1% in most studies vs ≤ 1% in the general population). AIDs with relatively high prevalence in the general population are also quite common in CSU patients, whereas those with low prevalence remain a rare finding in CSU. The rates of comorbidity in most studies were ≥ 1% for insulin-dependent diabetes mellitus, rheumatoid arthritis (RA), psoriasis and celiac disease (CD), ≥ 2% for Graves' disease, ≥ 3% for vitiligo, and ≥ 5% for pernicious anemia and Hashimoto's thyroiditis. Organ-specific AIDs are more prevalent in CSU than systemic (multiorgan or non organ-specific) AIDs. > 2% of CSU patients have autoimmune polyglandular syndromes encompassing autoimmune thyroid disease (ATD) and vitiligo or pernicious anemia. Antithyroid and antinuclear antibodies are the most prevalent AID-associated autoantibodies in CSU. > 15% of CSU patients have a positive family history for AIDs. The prevalence of urticarial rash in AID patients is > 1% in most studies. This rash is more prevalent in eosinophilic granulomatosis with polyangiitis, ATD, systemic lupus erythematosus, RA and CD. Conclusions CSU patients have an increased risk of AIDs, especially adult female patients and those with a positive family history and a genetic predisposition for AIDs, who should be screened for signs and symptoms of AIDs.