Orphanet Journal of Rare Diseases (Jul 2021)

Clinical features and treatment efficacy for IgG4-related thyroiditis

  • Xinxin Han,
  • Panpan Zhang,
  • Jieqiong Li,
  • Zheng Liu,
  • Hui Lu,
  • Xuan Luo,
  • Boju Pan,
  • Xiaolan Lian,
  • Xuejun Zeng,
  • Wen Zhang,
  • Xiaofeng Zeng

DOI
https://doi.org/10.1186/s13023-021-01942-x
Journal volume & issue
Vol. 16, no. 1
pp. 1 – 11

Abstract

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Abstract Purpose This study aimed to clarify the clinical features of and evaluate the treatment efficacy for IgG4-related thyroiditis. Methods Fourteen IgG4-related thyroiditis patients and 42 randomly matched IgG4-related disease (IgG4-RD) patients without thyroiditis in a prospective cohort at the Peking Union Medical College Hospital (PUMCH) were enrolled from 2011 to 2019. Patient demographics, clinical characteristics, laboratory parameters and treatment efficacy were analysed. Results The prevalence of IgG4-related thyroiditis in our cohort was 2.0%. The average patient age was 42.8 ± 14.9 years, and the male: female ratio was 1:1. Goiter (14, 100.0%), hard thyroid (14, 100.0%) and neck compression (5, 35.7%) were the most prevalent onset symptoms observed. IgG4-related thyroiditis was characterized by asymmetric diffuse thyroid enlargement on ultrasound. Thirteen (92.9%) patients had hypothyroidism, and all patients had significantly elevated circulating thyroid antibodies. Compared with patients without thyroiditis, patients with IgG4-related thyroiditis had less submandibular gland involvement and lacrimal gland involvement and lower serum IgG4 and T-IgE levels (P = 0.019, P = 0.022, P = 0.004, and P = 0.006, respectively) and more single-organ involvement (P = 0.011). After treatment, the symptoms were relieved, while the size of the thyroid gland did not change significantly, and levothyroxine as a supplemental therapy was still needed. Conclusions IgG4-related thyroiditis is a distinct subtype of IgG4-RD characterized by positive circulating thyroid antibodies and a high rate of hypothyroidism. Although compression symptoms could be relieved with treatment, the thyroid size did not change significantly, and the damage to thyroid function was often irreversible.