IgG4-related thyroiditis: a case report and review of literature

Summary A 55-year-old male, with a positive medical history for hypothyroidism, treated with stable doses for years was admitted with subacute thyroiditis and a feeling of pain and pressure in the neck. Laboratory tests showed decrease in TSH levels, elevated erythrocyte sedimentation rate, and very high antithyroid antibodies. Owing to enlarging goiter and exacerbation in the patient's complaints, he was operated with excision of a fibrotic and enlarged thyroid lobe. Elevated IgG4 plasma levels and high IgG4/IgG plasma cell ratio on immunohistochemistry led to the diagnosis of IgG4-mediated thyroiditis. We concluded that IgG4-thyroiditis and IgG4-related disease should be considered in all patients with an aggressive form of Hashimoto's thyroiditis. Learning points IgG4-related disease is a systemic disease that includes several syndromes; IgG4-related thyroiditis is one among them. IgG4-thyroiditis should be considered in all patients with an aggressive form of Hashimoto's thyroiditis. Patients with suspected IgG4-thyroiditis should have blood tested for IgG4/IgG ratio and appropriate immunohistochemical staining if possible.

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IgG4-related Hashimoto’s thyroiditis – A new variant of a well known disease

Arquivos Brasileiros de Endocrinologia & Metabologia ◽

10.1590/0004-2730000003283 ◽

2014 ◽

Vol 58 (8) ◽

pp. 862-868 ◽

Cited By ~ 7

Author(s):

Henrique Vara Luiz ◽

Diogo Gonçalves ◽

Tiago Nunes da Silva ◽

Isabel Nascimento ◽

Ana Ribeiro ◽

...

Keyword(s):

Hashimoto’S Thyroiditis ◽

Plasma Cells ◽

Hashimoto's Thyroiditis ◽

High Power Field ◽

Related Disease ◽

Igg4 Related Disease ◽

Inflammatory Parameters ◽

First Case ◽

New Variant ◽

Serum Igg4

Hashimoto’s thyroiditis (HT) has been characterized for many years as a well-defined clinicopathologic entity, but is now considered a heterogeneous disease. IgG4-related HT is a new subtype characterized by thyroid inflammation rich in IgG4-positive plasma cells and marked fibrosis. It may be part of the systemic IgG4-related disease. We report a case of a 56-year-old Portuguese man who presented with a one-month history of progressive neck swelling and dysphagia. Laboratory testing revealed increased inflammatory parameters, subclinical hypothyroidism and very high levels of thyroid autoantibodies. Cervical ultrasound (US) demonstrated an enlarged and heterogeneous thyroid gland and two hypoechoic nodules. US-guided fine needle aspiration cytology was consistent with lymphocytic thyroiditis. The patient was submitted to total thyroidectomy and microscopic examination identified typical findings of HT, marked fibrosis limited within the thyroid capsule and lymphoplasmacytic infiltration, with >50 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of >40%. After surgery, serum IgG4 concentration was high-normal. Symptoms relief and reduction in laboratory inflammatory parameters were noticed. Thyroid function is controlled with levothyroxine. To our knowledge we report the first case of IgG4-related HT in a non-Asian patient. We also perform a review of the literature regarding IgG4-related disease and IgG4-related HT. Our case highlights this new variant of the well known HT, and helps physicians in recognizing its main clinical features, allowing for proper diagnosis and treatment.

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Distinct histopathological features of Hashimoto’s thyroiditis with respect to IgG4-related disease

Modern Pathology ◽

10.1038/modpathol.2012.68 ◽

2012 ◽

Vol 25 (8) ◽

pp. 1086-1097 ◽

Cited By ~ 59

Author(s):

Yaqiong Li ◽

Gengyin Zhou ◽

Takashi Ozaki ◽

Eijun Nishihara ◽

Fumio Matsuzuka ◽

...

Keyword(s):

Hashimoto’S Thyroiditis ◽

Hashimoto's Thyroiditis ◽

Histopathological Features ◽

Related Disease ◽

Igg4 Related Disease

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Hashimoto’s thyroiditis manifesting as IGG4 related disease in a thyroid remnant following total thyroidectomy for grave’s disease

Pathology ◽

10.1097/01.pat.0000443608.87636.3b ◽

2014 ◽

Vol 46 ◽

pp. S79

Author(s):

M.N. Saxena ◽

A.A. Thomas

Keyword(s):

Total Thyroidectomy ◽

Hashimoto’S Thyroiditis ◽

Hashimoto's Thyroiditis ◽

Thyroid Remnant ◽

Related Disease ◽

Igg4 Related Disease ◽

Grave’S Disease ◽

Grave's Disease

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Hashimoto’s thyroiditis in a patient with IgG4 related disease

Endocrine Abstracts ◽

10.1530/endoabs.70.aep937 ◽

2020 ◽

Author(s):

Matali Nur Husnina ◽

Chong Pui Lin

Keyword(s):

Hashimoto’S Thyroiditis ◽

Hashimoto's Thyroiditis ◽

Related Disease ◽

Igg4 Related Disease

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IgG4-related disease of the thyroid gland

BMJ Case Reports ◽

10.1136/bcr-2020-238177 ◽

2021 ◽

Vol 14 (3) ◽

pp. e238177

Author(s):

Tânia Matos ◽

Margarida Mendes de Almeida ◽

Lucas Batista ◽

Sónia do Vale

Keyword(s):

Thyroid Gland ◽

Hashimoto’S Thyroiditis ◽

Plasma Cells ◽

Needle Aspiration ◽

Hashimoto's Thyroiditis ◽

Fine Needle ◽

Related Disease ◽

Igg4 Related Disease ◽

Cervical Swelling ◽

Reduced Mobility

IgG4-thyroid-related disease (TRD) represents an uncommon spectrum of diseases, with four subcategories established so far, IgG4-related Hashimoto’s thyroiditis, fibrosing variant of Hashimoto’s thyroiditis, Riedel’s thyroiditis and Graves disease with elevated IgG4 levels. We report the case of a 59-year-old woman presenting with painless cervical swelling and hypothyroidism. Thyroid gland was enlarged and distinctively very hard, with reduced mobility. Neck ultrasonography showed multiple nodularity and diffuse thyroid enlargement, which on CT scan conditioned slight deviation of the airway. Fine-needle aspiration of the biggest nodule was suggestive of lymphocytic thyroiditis. She developed compressive symptoms and was submitted to total thyroidectomy. Histology of the thyroid revealed extensive areas of fibrosis, oncocytic cells and lymphoplasmacytic infiltrates. Immunohistochemistry confirmed the predominance of IgG4-secreting plasma cells. IgG4-TRD is characterised by a rapidly progressive and destructive thyroiditis process. Typical presentation can often mimic malignancy; hence, an opportune recognition of IgG4-TRD may avoid unnecessary burdens.

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A small subgroup of Hashimoto’s thyroiditis is associated with IgG4-related disease

Virchows Archiv ◽

10.1007/s00428-015-1893-6 ◽

2015 ◽

Vol 468 (3) ◽

pp. 321-327 ◽

Cited By ~ 18

Author(s):

Friedrich Jokisch ◽

Irene Kleinlein ◽

Bernhard Haller ◽

Tanja Seehaus ◽

Heinrich Fuerst ◽

...

Keyword(s):

Hashimoto’S Thyroiditis ◽

Hashimoto's Thyroiditis ◽

Related Disease ◽

Igg4 Related Disease ◽

Small Subgroup

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Immune Disorders in Hashimoto’s Thyroiditis: What Do We Know So Far?

Journal of Immunology Research ◽

10.1155/2015/979167 ◽

2015 ◽

Vol 2015 ◽

pp. 1-8 ◽

Cited By ~ 61

Author(s):

Aleksandra Pyzik ◽

Ewelina Grywalska ◽

Beata Matyjaszek-Matuszek ◽

Jacek Roliński

Keyword(s):

Hashimoto’S Thyroiditis ◽

T Regulatory Cells ◽

Hashimoto Thyroiditis ◽

Hashimoto's Thyroiditis ◽

Regulatory Cells ◽

Review Of Literature ◽

Immune Disorders ◽

Cd4 Cells ◽

Immune Defect

This review of literature attempts to identify the factors that are involved in the pathogenesis of Hashimoto thyroiditis, an immune defect in an individual with genetic susceptibility accompanied with environmental factors. The frequency of Hashimoto’s disease is a growing trend and among Caucasians it is estimated at approximately 5%. The dysfunction of the gland may be clinically evident (0.1–2% of the population) or subclinical (10–15%). The pathology is diagnosed five to ten times more often in women than men and its incidence increases with the age (the peak of the number of cases is between 45 and 65); however, it can also be diagnosed in children. The pathogenesis of Hashimoto’s thyroiditis is still not fully comprehended. In the etiology of Hashimoto thyroiditis excessively stimulated T CD4+ cells are known to play the most important role. Recent research has demonstrated an increasing role of newly discovered cells such as Th17 (CD4+IL-17+) or T regulatory cells (CD4+CD25+highFoxP3+) in the induction of autoimmune disorders. The process of programmed cell death also plays an equally important role in the pathogenesis and the development of hypothyroidism.

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Rituximab treatment for isolated IgG4-related hypophysitis in a teenage female

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-18-0135 ◽

2018 ◽

Vol 2018 ◽

Cited By ~ 2

Author(s):

Danielle R Bullock ◽

Bradley S Miller ◽

H Brent Clark ◽

Patricia M Hobday

Keyword(s):

Endocrine Function ◽

List Type ◽

Pituitary Mass ◽

Related Disease ◽

Pituitary Dysfunction ◽

Igg4 Related Disease ◽

Pituitary Disease ◽

Appropriate Treatment ◽

Organ Systems ◽

Progression Of Disease

Summary IgG4-related hypophysitis is an important diagnostic consideration in patients with a pituitary mass or pituitary dysfunction and can initially present with headaches, visual field deficits and/or endocrine dysfunction. Isolated IgG4-related pituitary disease is rare, with most cases of IgG4-related disease involving additional organ systems. We report the case of a teenage female patient with isolated IgG4-related hypophysitis, diagnosed after initially presenting with headaches. Our patient had no presenting endocrinologic abnormalities. She was treated with surgical resection, prednisolone and rituximab with no further progression of disease and sustained normal endocrine function. This case, the youngest described patient with isolated IgG4-related hypophysitis and uniquely lacking endocrinologic abnormalities, adds to the limited reports of isolated pituitary disease. The use of rituximab for isolated pituitary disease has never been described. While IgG4-related hypophysitis has been increasingly recognized, substantial evidence concerning the appropriate treatment and follow-up of these patients is largely lacking. Learning points: IgG4-related hypophysitis most often occurs in the setting of additional organ involvement but can be an isolated finding. This diagnosis should therefore be considered in a patient presenting with pituitary abnormalities. Most patients with IgG4-related hypophysitis will have abnormal pituitary function, but normal functioning does not exclude this diagnosis. Corticosteroids have been the mainstay of therapy for IgG4-related disease, with other immunosuppressive regimens being reserved for refractory cases. Further research is needed to understand the effectiveness of corticosteroid-sparing regimens and whether there is utility in using these agents as first-line therapies.

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