Riedel's Thyroiditis Research Articles

An uncommon infiltrative disease of thyroid: Riedel's thyroiditis

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

18F-FDG PET-CT usefulness in extra-pancreatic involvement in IgG4 related diseases

IgG4-related diseases are a group of recently identified entities that include disorders that were previously known by other names, such as Mikulicz disease, Küttner's tumor, Riedel thyroiditis, among others, as well as some new ones described in the last years. These pathologies are a challenge for the medical community in terms of diagnosis and characterization due to their wide spectrum of clinical presentation. Functional imaging can provide a new approach to the comprehension of physiopathology, staging and targeting site of biopsy of IgG4-related diseases. In this clinical note, we describe five patients who underwent ¹⁸F-FDG PET-CT and correlate their findings with previous reports.

Elevated Serum Immunoglobulin G4 Levels in Patients with Graves' Disease and Their Clinical Implications

Immunoglobulin G4-related disease (IgG4-RD) is a new clinical entity that affects various organs with increased IgG4 positive plasmacytes and progressive fibrosis. While IgG4-RDs in association with Hashimoto's thyroiditis or Riedel's thyroiditis have been reported, the relationship between IgG4-RD and Graves' disease (GD) is yet unknown. To elucidate the relation of GD to IgG4-RD, serum IgG4 levels and their clinical implications in patients with GD were investigated. In this prospective study, serum IgG4 levels were measured in 109 patients with GD and classified into two groups according to the comprehensive diagnostic criteria of IgG4-RD previously established: (i) GD with elevated-IgG4 levels (≥ 135 mg/dL), and (ii) GD with nonelevated IgG4 (<135 mg/dL). Seven out of 109 patients with GD (6.4%) had elevated serum IgG4 levels [mean ± standard deviation (range): 175.0 ± 44.5 (136-266) mg/dL] and elevated ratios of IgG4/IgG [12.7 ± 4.5% (7.6%-21.2%)]. The remaining patients with GD had serum IgG4 levels and IgG4/IgG ratios of 39.6 ± 27.6 (3-132) mg/dL and 3.2 ± 2.2% (0.3%-11.5%), respectively. Ages in the elevated IgG4 group were significantly higher than those of the nonelevated IgG4 group: 54.7 ± 6.2 versus 43.4 ± 15.4 years, respectively. Ultrasound examinations revealed that the elevated IgG4 group had significantly increased hypoechogenic areas in the thyroid in comparison to the nonelevated IgG4 group (low echo scoring: 1.66 ± 0.81 vs. 0.61 ± 0.89, respectively). In the correlation analysis, TSAb (rs=0.385, n=42) titers were significantly correlated with se rum IgG4 levels, while they were not significantly different between the two groups. In the elevated IgG4 group, symptoms were controllable with a small dose of antithyroidal drug (ATD; n=4), a combination treatment with ATD and L-T4 (n=1), or L-T4 administration only one year after the first visit (n=2). A small portion of GD patients harbored elevated serum IgG4 levels. They were older, had increased hypoechoic areas in the thyroid, and appeared to be responsive or prone to be hypothyroid after ATD treatment. Thus, the present study suggests the presence of a novel subtype of GD. Measuring serum IgG4 levels may help to distinguish this new entity and provide potential therapeutic options for GD.

Pharyngolaryngeal Reflux Simulating Vocal Fold Paralysis Hoarseness following Total Thyroidectomy

Objectives: Systemic multifocal fibrosclerosis is a syndrome characterized by the presence of fibrosis in various locations, most often retroperitoneal, mediastinal, and thyroid (Riedel thyroiditis). The fibrosis in the thyroid tissue can extend to the laryngeal cartilages and muscles, leading to respiratory distress. A thyroidectomy is, in these cases, an intervention to consider. Recognize potential “rare” causes of hoarseness following thyroid surgery. Method: Hoarseness case study. A 69 year old man presented to the ear, nose, and throat (ENT) consultation with a history of hoarseness and aspiration following total thyroidectomy for Riedel’s thyroiditis. Physical examination revealed exuberant pharyngolaryngeal reflux with consequent edema and redundancy of arytenoid mucosa with simetric mobility of the vocal folds. Results: Worsening dysphonia after total thyroidectomy, particularly in the context of Riedel thyroiditis, leads to suspicion of iatrogenic vocal fold paresis. The vocal fold preserved mobility, associated with signs of exuberant pharyngolaryngeal reflux with extensive mucosal damage, reveals an unsuspected cause of hoarseness. The authors postulate that post-operatory changes in esophagic sphincter pressure may have triggered reflux. Conclusions: Rare causes of hoarseness following thyroid surgery should be considered in order to promptly establish a correct treatment plan.

IgG4-related disease

The term “IgG4-related disease” encompasses several disorders described many years ago under various designations depending on the organ or system involved (e.g., Mikulicz syndrome, Riedel's thyroiditis, and retroperitoneal fibrosis). The clinical presentation varies widely, as one or more organs may be affected, usually in the same region of the body and either synchronously or metachronously. The main targets are the pancreas, bile ducts, salivary glands, lachrymal glands, mediastinal lymph nodes, and retroperitoneum. IgG4-related disease is rare, with an estimated incidence of 0.2 to 1/100 000 in Japan and no available incidence data in occidental countries. Men older than 50 years are predominantly affected. Serum IgG4 levels are often greater than 1.35g/L. Enlargement of the involved organs, which may be pseudotumoral, is due to a combination of infiltration by T cells and IgG4-expressing plasma cells, storiform fibrosis, and obliterative thrombophlebitis. Glucocorticoid therapy is effective but may be followed by relapses requiring the use of immunomodulating agents such as azathioprine, methotrexate and, more recently, rituximab. IgG4-related disease is not an autoimmune condition related to IgG4 autoantibodies, and neither does it involve immune complexes. Specific joint involvement has been reported in a very small number of patients.

Novel Use of Rituximab in a Case of Riedel's Thyroiditis Refractory to Glucocorticoids and Tamoxifen

A 42-year-old woman presented with a rapidly enlarging right-sided thyroid mass and underwent hemithyroidectomy. Riedel's thyroiditis was only diagnosed upon surgical decompression of the right carotid artery 2 years later. She became more symptomatic as Riedel's thyroiditis progressed, and mediastinal fibrosclerosis developed over the next 12 months. Oral prednisolone failed to improve her condition, and she was commenced on tamoxifen. Despite initial improvement, her symptoms recurred 2 years later, mainly arising from compression of the trachea and esophagus at the thoracic inlet. Fluorodeoxyglucose positron emission tomographic scan showed locally advanced active invasive fibrosclerosis in the neck and mediastinum. An elevated activin-A level of 218 pg/mL was consistent with active inflammation. IgG subtypes (including IgG4) were normal. Two courses of iv methylprednisolone were given but only produced transient improvement. Subsequently, the patient received 3 doses of i.v. rituximab at monthly intervals and had prompt sustained symptomatic improvement. Activin-A level decreased to 122 pg/mL 10 months after rituximab therapy. Fluorodeoxyglucose positron emission tomographic scan 6 weeks after therapy showed reduction in inflammation. A further scan at 10 months demonstrated ongoing response to rituximab. This is a case of refractory Riedel's thyroiditis with symptomatic, biochemical, and radiological improvement that has persisted 14 months after rituximab. The likelihood and duration of response to rituximab in Riedel's thyroiditis requires further study.

Fatal Neck Necrotizing Cellulitis in a Patient with Riedel's Thyroiditis

ThyroidVol. 23, No. 7 Letters to the EditorFatal Neck Necrotizing Cellulitis in a Patient with Riedel's ThyroiditisAgnès Dupret-Bories, Philippe Caron, Isabelle Rouquette, Benjamin Vairel, and Sébastien VergezAgnès Dupret-BoriesDepartment of Otorhinolaryngology and Head and Neck Surgery, Hautepierre University Hospital Center, Strasbourg, France.Search for more papers by this author, Philippe CaronDepartment of Endocrinology and Metabolic Disease (Cardiovascular and Metabolism Division), Larrey University Hospital Center, Toulouse, France.Search for more papers by this author, Isabelle RouquetteDepartment of Pathology and Laboratory Medicine, Larrey University Hospital Center, Toulouse, France.Search for more papers by this author, Benjamin VairelDepartment of Otorhinolaryngology and Head and Neck Surgery, Larrey University Hospital Center, Toulouse, France.Search for more papers by this author, and Sébastien VergezDepartment of Otorhinolaryngology and Head and Neck Surgery, Larrey University Hospital Center, Toulouse, France.Search for more papers by this authorPublished Online:8 Jul 2013https://doi.org/10.1089/thy.2012.0569AboutSectionsView articleView Full TextPDF/EPUB ToolsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail View article"Fatal Neck Necrotizing Cellulitis in a Patient with Riedel's Thyroiditis." , 23(7), pp. 904–905FiguresReferencesRelatedDetailsCited byRiedel Thyroiditis20 July 2020 | The Journal of Clinical Endocrinology & Metabolism, Vol. 105, No. 9Thyroiditis15 March 2016 Volume 23Issue 7Jul 2013 InformationCopyright 2013, Mary Ann Liebert, Inc.To cite this article:Agnès Dupret-Bories, Philippe Caron, Isabelle Rouquette, Benjamin Vairel, and Sébastien Vergez.Fatal Neck Necrotizing Cellulitis in a Patient with Riedel's Thyroiditis.Thyroid.Jul 2013.904-905.http://doi.org/10.1089/thy.2012.0569Published in Volume: 23 Issue 7: July 8, 2013Online Ahead of Print:June 21, 2013Online Ahead of Editing: March 25, 2013PDF download

AB1234 Clinical characteristics of IgG4-related orbital pseudo-tumor

BackgroundIgG4-related disease (IgG4-RD) is a new emerging disease entity of unknown etiology with multiorgan involvement. IgG4-RD includes a wide variety of diseases, including Mikulicz’s disease (MD), autoimmune pancreatitis (AIP), hypophysitis,...

Vasovagal reflex emergency caused by Riedel's thyroiditis: A case report and review of the literature

Riedel's thyroiditis is a rare type of chronic thyroiditis, associated with fibroinflammatory process and invasion into surrounding tissues, leading to compressive symptoms. A 45-year-old man had a left thyroid mass, presenting with hypotension and bradycardia many times. He was diagnosed with vasovagal reflex caused by cervical vessel compression due to a thyroid lesion. We performed the emergency operation, and most of the left thyroid was removed to relieve the compression on cervical vessels. The result of pathology proved to be Riedel's thyroiditis. The vasovagal reflex did not occur any more during the 28-month follow up, except on the 3(rd) day after the surgery. Six months after the thyroidectomy, the patient was found to have retroperitoneal fibrosis, diagnosed by biopsy during a laparotomy for biliary disease. Riedel's thyroiditis can lead to a vasovagal episode and might not be a primary thyroid disease but rather a manifestation of the systemic disorder, multifocal fibrosclerosis.

Fibrose(s) rétropéritonéale(s) : stratégie diagnostique, pathologies associées et suivi à long terme d’une cohorte française

PurposeRetroperitoneal fibrosis (RPF) is a rare disease with an expanding etiologic spectrum. We aimed to analyze non-invasive diagnosis strategy, associated disorders, monitoring, treatment and prognosis. MethodsRetrospective cohort study in a single tertiary center. ResultsEighteen RPF cases (11 males) followed between 1996 and 2009 were reviewed. Blood CRP level was high in all cases before treatment. CT scan, associated or not with MRI or 18-FDG PET-scan, confirmed the diagnosis in 15 patients. Histological analysis of a surgical biopsy specimen was performed in only three cases. Ten patients suffered retroperitoneal fibrosis secondary to systemic vasculitis (granulomatosis with polyangeitis, n=1, Takayasu aortitis, n=2), systemic fibrosis with Riedel thyroiditis (n=1) and atheromatous periaortitis (n=6). Fifteen patients were treated with corticosteroids with a mean treatment duration of 60 months (12–228). Dependency to corticosteroids was recorded in ten patients. Patients with fibrosis related to vasculitis were younger, had a higher CRP level, more frequent corticosteroid dependency and a higher relapse rate. Relapses were successfully treated with steroids. Immunosuppressive treatment was only prescribed in the setting of systemic vasculitis. No patient died, after a 6±2 years follow-up. Late relapses could occur, sometimes years after steroid therapy cessation. ConclusionIn our study, RPF occurred as a secondary disorder in 60% of the cases. Disease extension, relapse rate and treatment response varied according to the underlying cause of RPF, pleading for an extensive and systematic initial assessment. Since no death or end-stage renal insufficiency was observed, RPF might be considered as a steroid-sensitive and benign disorder.

Mucosa-associated lymphoid tissue lymphoma of the thyroid with abundant IgG4-positive plasma cells

A case of thyroidal mucosa-associated lymphoid tissue (MALT) lymphoma mimicking IgG4-related disease is described. A 54-year-old male presented with acute swelling of the anterior neck. Anaplastic thyroid carcinoma (ATC), malignant lymphoma (ML), or acute deterioration of Hashimoto's thyroiditis were initially suspected, and an emergent tracheostomy was required for progressive airway obstruction; a simultaneous biopsy from the thyroid tissue was performed. Histopathologically, the lesion consisted of sclerotic fibrosis and diffuse and dense infiltration by small lymphoid cells without atypia and plasma cells, many of which were IgG4-positive. Blood examination also revealed high serum IgG4 levels. Riedel's thyroiditis was suspected. However, despite medical treatments, a firm swelling of the thyroid still remained. In an in situ hybridization study, IgG4-negative plasma cells showed immunoglobulin light-chain restriction (κ-monotype), and immunoglobulin heavy (IgH) chain gene monoclonal re-arrangement was detected by polymerase chain reaction. The lesion was finally diagnosed as MALT lymphoma. When IgG4-related disease is suspected, it is important to thoroughly exclude other possibilities.

Riedel Thyroiditis in a Patient with Graves Disease

Riedel's thyroiditis is a rare form of infiltrative and inflammatory disease of the thyroid gland and can be associated with systemic fibrotic processes, Hashimoto thyroiditis and Graves disease. Riedel thyroiditis in combination with Graves disease however, is very rare. A 57-year-old woman with a past medical history significant for Graves disease diagnosed 30 years ago presented with an enlarging neck mass and voice changes. Due to suspicion of malignancy, thyroidectomy was performed. Histopathologic examination revealed Riedel thyroiditis. To our knowledge, the association of Riedel thyroiditis with Graves disease has not yet been reported in our country. Here we report a patient with Riedel thyroiditis evolved from antecedent Graves disease.

Case of concurrent Riedel's thyroiditis, acute suppurative thyroiditis, and micropapillary carcinoma

Riedel's thyroiditis (RT) is a rare chronic inflammatory disease of the thyroid gland. It is characterized by a fibroinflammatory process that partially destroys the gland and extends into adjacent neck structures. Its clinical manifestation can mask an accompanying thyroid neoplasm and can mimic invasive thyroid carcinoma. Therefore, diagnosis can be difficult prior to surgical removal of the thyroid, and histopathologic examination of the thyroid is necessary for a definite diagnosis. The concurrent presence of RT and other thyroid diseases has been reported. However, to our knowledge, the association of RT with acute suppurative thyroiditis and micropapillary carcinoma has not been reported. We report a rare case of concurrent RT, acute suppurative thyroiditis, and micropapillary carcinoma in a 48-year-old patient.

IgG4-Related Disease

IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by an elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4-RD encompasses a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), hypophysitis, Riedel thyroiditis, tubulointerstitial nephritis, prostatitis, retroperitoneal fibrosis, inflammatory aortic aneurysm and inflammatory pseudotumor. However, like a crow flying on a dark night, IgG4-RD passed through the history of medicine, until the concept of an overarching of these systemic disorders was established in Japan at the beginning of the present century. In addition, most clinical practitioners are not yet familiar with IgG4-RD, and thus one cannot diagnose what one does not know. The purpose of the book [1] is to raise awareness of this disease and its diagnostic pitfalls. Another goal was to delineate the characteristic features of individual organs in IgG4-RD radiologically and histopathologically with many color photographs. Most of the authors are members of the IgG4 team of the Japanese the Ministry of Health, Labor and Welfare (MHLW), and experts who have published numerous important papers in the field. Thus, this book will be useful to physicians in various disciplines such as gastroenterology, rheumatology, ophthalmology, otolaryngology, urology, hematology, respiratory medicine and oral medicine, not only as a textbook but also as an authoritative and comprehensive reference work.

A misdiagnosed Riedel's thyroiditis successfully treated by thyroidectomy and tamoxifen

Riedel's thyroiditis, known as invasive fibrous thyroiditis, is a very rare form of chronic thyroiditis. It is hard to make the diagnosis without surgical biopsy. We present a case of Riedel's thyroiditis in a 52-year-old female with past history of Hashimoto's thyroiditis. She suffered from bilateral neck pain, which radiated to both lower jaws. The erythrocyte sedimentation rate was 125mm/hour. Subacute thyroiditis superimposed on Hashimoto's thyroiditis was diagnosed and treated with steroid. However the response was poor and she had a history of severe peptic ulcer. To avoid inducing the peptic ulcer by steroid, she received bilateral subtotal thyroidectomy. During surgery, the thyroid had severe adhesion to surrounding soft tissue and the pathology showed Riedel's thyroiditis. The neck pain improved after thyroidectomy. Tamoxifen has been given for 8 months and the size of remnant thyroid decreased to 8mm. We concluded that combined thyroidectomy and tamoxifen successfully cured a patient with Riedel's thyroiditis.

IgG4-related Disease of the Head and Neck: CT and MR Imaging Manifestations

Immunoglobulin G4 (IgG4)-related disease is a recently established systemic disease that commonly involves the head and neck, including the salivary glands, lacrimal glands, orbits, thyroid gland, lymph nodes, sinonasal cavities, pituitary gland, and larynx. Although the definitive diagnosis of IgG4-related disease requires histopathologic analysis, elevated serum IgG4 levels are helpful in making the diagnosis. Because of the proposed clinical diagnostic criteria for this disease, cross-sectional imaging modalities such as computed tomography (CT) and magnetic resonance (MR) imaging play an important diagnostic role. CT and MR imaging findings of IgG4-related disease are usually nonspecific. At CT, involved organs may demonstrate enlargement or decreased attenuation; at T2-weighted MR imaging, they may have relatively low signal intensity owing to their increased cellularity and amount of fibrosis. Some pathologic entities involving the head and neck are now considered to be part of the IgG4-related disease spectrum, including idiopathic orbital inflammatory syndrome (inflammatory pseudotumor), orbital lymphoid hyperplasia, Mikulicz disease, Küttner tumor, Hashimoto thyroiditis, Riedel thyroiditis, and pituitary hypophysitis. Because involvement of multiple sites is common in IgG4-related disease, radiologists should be familiar with manifestations of this systemic process outside the head and neck, in organs such as the pancreas, bile ducts, gallbladder, kidneys, retroperitoneum, mesentery, lungs, gastrointestinal tract, and blood vessels. Moreover, IgG4-related disease usually demonstrates a dramatic response to corticosteroid therapy, and radiologists should be familiar with its clinical and imaging manifestations to avoid a delay in diagnosis or unnecessary invasive interventions.

Nodular sclerosing Hodgkin's disease mimicking Riedel's invasive fibrous thyroiditis

Riedel's thyroiditis appears in the form of a hard cervical mass with rapid onset, and it is associated with extensive fibrosis that compresses nearby structures, such as the trachea and supra-aortic vessels; its diagnosis is essentially histopathological. Although its histological characteristics have been well established, there are some diagnostic pitfalls. We report here the case of a 37-year-old woman, with clinical and histopathological data suggesting Riedel's disease. Fibrosis regressed after treatment with corticosteroids, relieving the compressed airways. However, in contrast with the latest knowledge on this disease, the IgG4 serum levels were consistently normal, and positron emission tomography in search of extensive fibrosis revealed an abnormal metabolic activity of the bone marrow. The final diagnosis revised by the histopathologist was that of nodular sclerosing Hodgkin's lymphoma. This case allows us to review the diagnostic approach when facing a thyroid mass with extremely rapid evolution.

Tiroiditis

The term thyroiditis encompasses entities that may result from an acute illness with severe thyroid pain (subacute granulomatous thyroiditis or infectious), to situations where the clinic without inflammation depends on the presence of goiter and/or thyroid dysfunction (thyroiditis, fibrous thyroiditis or painless). The subacute thyroiditis, subacute granulomatous thyroiditis as the silent and postpartum thyroiditis, usually present with an initial hyperthyroid phase, a period subsequent hypothyroid and finally a full recovery. Treatment is symptomatic with beta-blockers in hyperthyroid phase and levothyroxine if hypothyroidism appears. The suppurative thyroiditis due to acute infection requires of aspiration for drainage and to identify the organism and the antibiotic selection. Chronic lymphocytic thyroiditis is associated with positive antithyroid antibodies and usually evolves to hypothyroidism which require treatment with levothyroxine. Riedel's fibrosing thyroiditis is a rare entity that must be differentiated from malignant entities whose treatment is surgical.

Riedel's Thyroiditis with Increased IgG4 Plasma Cells: Evidence for an Underlying IgG4-Related Sclerosing Disease?

Riedel's thyroiditis (RT) is a very rare chronic fibrosing disorder of unknown etiology that is often associated with multifocal fibrosclerosis (MFS). Immunoglobulin (Ig) G4-related sclerosing disease (IgG4-RSD), a new clinico-pathological entity also associated with MFS, is characterized by IgG4+ plasma cell infiltration and fibrosis in one or more organs. Although the association of RT and IgG4-RSD has been suggested, it has seldom been studied or reported. We report a classical case of RT with serological (IgG4 levels) and immunohistochemical (IgG and IgG4) assessment, in search of an underlying IgG4-RSD. The patient was a 57-year-old woman who underwent a subtotal thyroidectomy for a long-standing goiter with a rapidly enlarging isthmic nodule. Histopathological examination of the surgical specimen revealed all of the morphological features of RT and IgG4-RSD, including partial fibrosis of the thyroid gland with destruction of the thyroid follicular architecture; obliterative phlebitis; and a mixed infiltrate composed of lymphocytes, eosinophils, and plasma cells. The fibro-inflammatory process extended beyond the thyroid capsule into the surrounding tissues. Immunohistochemical examination revealed approximately 70 IgG4+ plasma cells per high power field (HPF) with an IgG4/IgG ratio of 35%. Although serum levels of IgG4 were normal (20 mg/dL), total IgG levels were slightly elevated (1370 mg/dL). There was no evidence of involvement of other organs at the time of RT diagnosis. The morphological similarities between RT and IgG4-RSD suggest that these entities are closely related. Therefore, RT with increased IgG4+ plasma cells, with or without elevated IgG4 serum levels, may represent the first clinical manifestation of an underlying IgG4-RSD. However, due to the rarity of both conditions and the limited specificity and sensitivity of both IgG4 serum levels and IgG/IgG4 immunohistochemistry in the diagnosis of IgG4-RSD, further studies are needed to verify this hypothesis.

Hashimoto Thyroiditis

More than a century has passed since the first description of Hashimoto thyroiditis (HT) as a clinicopathologic entity. HT is an autoimmune disease in which a breakdown of immune tolerance is caused by interplay of a variety of immunologic, genetic, and environmental factors. Thyrocyte injury resulting from environmental factors results in expression of new or hidden epitopes that leads to proliferation of autoreactive T and B cells. Infiltration of thyroid by these cells results in HT. In addition to the usual type of HT, several variants such as the fibrous type and Riedal thyroiditis are also recognized. The most recently recognized variant is immunoglobulin G4(+) HT, which may occur as isolated thyroid limited disease or as part of a generalized Ig4-related sclerosing disease. The relationship between HT and Riedel thyroiditis remains unclear; however, recent evidence seems to suggest that it may also be part of the spectrum of Ig4-related sclerosing disease. HT is frequently associated with papillary thyroid carcinoma and may indeed be a risk factor for developing this type of cancer. The relationship between thyroid lymphoma and HT on the other hand appears well established.