Abstract

PurposeThis study aimed to clarify the clinical features of and evaluate the treatment efficacy for IgG4-related thyroiditis.MethodsFourteen 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.ResultsThe 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.ConclusionsIgG4-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.

Highlights

  • IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder [1, 2]

  • Patient enrolment In our prospective cohort study of IgG4-RD carried out in the Peking Union Medical College Hospital, 710 IgG4related disease patients fulfilling the 2011 comprehensive diagnostic criteria were enrolled from January 2011 to November 2019

  • A patient was diagnosed with IgG4-related thyroiditis if he/she fulfilled the diagnostic criteria and had a hard thyroid enlarged in size confirmed by a physical examination and imaging

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Summary

Introduction

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder [1, 2]. IgG4-RD is characterized by tumefactive swelling of affected organs, elevated serum IgG4, dense lymphocyte infiltration and IgG4positive plasma cells in tissues [3, 4]. Accumulating evidence has revealed that a small population of patients with thyroiditis have elevated serum IgG4 and tissue infiltration of IgG4-positive plasma cells [7,8,9]. As one of the less commonly involved organs of IgG4RD, IgG4-related thyroiditis mainly includes Hashimoto’s thyroiditis (HT) and Riedel’s thyroiditis (RT) [7, 11]. Awareness of this distinct entity may help clinicians guide treatment strategies. The management of IgG4-related thyroiditis could be challenging due to the lack of available information about this disease [15]

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