Proposal of diagnostic criteria for IgG4-related thyroid disease

Takeshima, Ken; Li, Yaqiong; Kakudo, Kennichi; Hirokawa, Mitsuyoshi; Nishihara, Eijun; Shimatsu, Akira; Takahashi, Yutaka; Akamizu, Takashi

doi:10.1507/endocrj.ej20-0557

Cited by 21 publications

(21 citation statements)

References 38 publications

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“…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]. The thyroid gland is recognized as an entity of IgG4-RD, although the spectrum of IgG4-related thyroiditis remains to be defined [10].…”

Section: Introductionmentioning

confidence: 99%

Clinical features and treatment efficacy for IgG4-related thyroiditis

Han

Zhang

et al. 2021

Orphanet J Rare Dis

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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.

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Section: Introductionmentioning

confidence: 99%

Clinical features and treatment efficacy for IgG4-related thyroiditis

Han

Zhang

et al. 2021

Orphanet J Rare Dis

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“…In both studies, the "high number of IgG4+ plasma cells" was defined as >10 cells in a high power field (HPF) and a ratio of IgG4(+) cells to IgG(+) cells > 40%, which is the most common criterium used in IgG4-RD [81]. However, some authors suggest using organ-specific criteria, in the case of the thyroid gland as above 20 IgG4(+) plasma cells in HPF and an IgG4(+) to IgG(+) plasma cell ratio > 30% [82]. None of the abovementioned studies found other characteristic features of IgG4-RD like storiform fibrosis or obliterative phlebitis in the analyzed thyroid gland specimens, while fibrosis was present in only one case in the cohort analyzed by Nishihara et al [80].…”

Section: Role Of Igg4 In Graves' Disease and Orbitopathymentioning

confidence: 99%

IgG4 as a Biomarker in Graves’ Orbitopathy

Olejarz

Szczepanek‐Parulska

Dadej

et al. 2021

Mediators of Inflammation

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Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disorder associated with fibrosis and abundant tissue lymphoplasmacytic infiltrations. It typically affects the pancreas, the salivary glands, and the retroperitoneal space. However, it might also involve multiple other organs, including the orbit and the thyroid. Recent studies have suggested that IgG4 plays a role in the pathophysiology of autoimmune thyroid diseases. This ultimately led to the establishment of new clinical entities called IgG4-related thyroid disease and thyroid disease with an elevation of IgG4. The aim of this paper is to describe the pathophysiological, histopathological, and clinical features of Graves’ Disease (GD) and Graves’ Orbitopathy (GO) with elevated IgG4 levels. Multiple studies have demonstrated higher IgG4 serum concentrations in GD patients than in healthy euthyroid controls. Depending on the studied population, elevated serum IgG4 levels occur in 6.4-23% (average: 10.3%) of all patients with GD, 8.3-37.5% (average: 17.6%) of patients with GO, and 0-9.8% (average: 5.4%) of patients with GD without GO, while GO patients comprise 37.5-100% (average: 65.8%) of all GD patients with elevated IgG4 levels. Characteristic features of GD with elevated IgG4 levels include lower echogenicity of the thyroid gland on ultrasound examination, peripheral blood eosinophilia, higher prevalence of orbitopathy, and better response to antithyroid drugs with a tendency to develop hypothyroidism when compared to patients with GD and normal levels of IgG4. Typical signs of GO accompanied by increased concentration of IgG4 include younger age at diagnosis, and more severe course of the disease with a higher Clinical Activity Score (CAS).. We strongly recommend considering the diagnosis of GO with elevated IgG4 in patients with an established diagnosis of GD, elevated serum IgG4 levels, and clinical features of ophthalmic disease overlapping with those of IgG4-related orbital disease.

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“…IgG4-related disease (IgG4-RD) is a multi-organ disorder characterized by high serum IgG4 levels and massive lesions with severe fibrosis and infiltration of IgG4-positive plasma cells. The typically affected organs include the pancreas, lacrimal glands, salivary gland, thyroid, lungs, kidneys, and the retroperitoneum; the pituitary gland can also be affected ( 1 , 2 ).…”

Section: Introductionmentioning

confidence: 99%

Deterioration of pituitary function without relapse after steroid therapy for IgG4-related hypophysitis

Nishi

Takeshima

Morita

et al. 2021

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary IgG4-related hypophysitis is an autoimmune hypophysitis associated with IgG4-related disease. Swelling of the pituitary gland is responsive to steroid therapy, but the prognosis of pituitary function after the treatment remains unclear. The present case implies that transiently improved pituitary function can re-worsen during long-term follow-up in IgG4-related hypophysitis. A 71-year-old male patient with IgG4-related hypophysitis visited a nearby hospital with malaise, anorexia, and polyuria. Pituitary dysfunction was suspected, so he was referred to our hospital for further examination. Imaging studies and laboratory data showed swelling of the pituitary gland and panhypopituitarism, which dramatically improved following steroid therapy. There was no evidence of relapsing IgG4-related disease during prednisolone tapering. Pituitary function was examined after 4 years under treatment with low-dose prednisolone; surprisingly, anterior pituitary function had worsened again. Our case suggests a need for continuous monitoring of pituitary function after steroid therapy for IgG4-related hypophysitis. This report illustrates the natural course of pituitary function in IgG4-related hypophysitis and may be informative when considering the introduction of steroid therapy. Learning points Steroid therapy is an effective first-line therapy for pituitary dysfunction and pituitary swelling in IgG4-related hypophysitis. Pituitary function can worsen again during follow-up, despite transient improvement after steroid therapy in IgG4-related hypophysitis. Continuous monitoring of pituitary function is necessary for IgG4-related hypophysitis, regardless of disease activity.

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Proposal of diagnostic criteria for IgG4-related thyroid disease

Cited by 21 publications

References 38 publications

Clinical features and treatment efficacy for IgG4-related thyroiditis

Clinical features and treatment efficacy for IgG4-related thyroiditis

IgG4 as a Biomarker in Graves’ Orbitopathy

Deterioration of pituitary function without relapse after steroid therapy for IgG4-related hypophysitis

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