Invasive Fibrous Thyroiditis Research Articles

Life-threatening airway obstruction by Riedel's thyroiditis: a rare presentation and diagnostic dilemma.

Riedel's thyroiditis is the rarest form of thyroiditis, occasionally resulting in rapid thyroid enlargement and potential tracheal obstruction. Here, we detail the case of an 81-year-old woman with a medical history including Hodgkin lymphoma, Hashimoto's thyroiditis, and multinodular goiter. She presented to the emergency room with stridor, cervical swelling, and breathing difficulties for over 2 days. CT scans revealed substantial thyroid enlargement causing significant glottal and tracheal compression, to a minimum tracheal diameter of 7 mm. Due to the severity of the compressive symptoms, orotracheal intubation and mechanical ventilation were deemed necessary. Surprisingly, despite the initial suspicion of malignancy given the rapid growth in the elderly, subsequent cytological and histological evaluations indicated a benign form of invasive fibrous thyroiditis - Riedel's thyroiditis. Although surgical intervention was advised, the patient declined and opted for endobronchial treatment with a prosthetic stent and subsequent treatment with systemic glucocorticoids. Following successful treatment, she was discharged within a week and resumed normal activities without respiratory distress. This case is noteworthy for its rapid benign mass growth, rare emergent presentation, and the patient's advanced age. The rapid enlargement of the thyroid gland in elderly patients poses a diagnostic challenge, stemming from the higher occurrence of aggressive thyroid carcinomas. Despite the clinical presentation, a comprehensive diagnostic workup, including fine-needle aspiration and core-needle biopsy, is crucial for accurately distinguishing between benign and malignant causes of thyroid nodule enlargement. This case report illustrates diverse treatment options for Riedel's thyroiditis, and the importance of individualized treatment plans based on the degree of airway obstruction, patient preferences, and response to initial interventions. Clinicians should contemplate the inclusion of glucocorticoids in the therapeutic regimen for Riedel's thyroiditis, particularly in cases where surgical intervention is not feasible or declined by the patient.

Een harde schildkliermassa is niet altijd maligne

A hard thyroid mass: not always a malignancy In this article, the medical history of a patient with a hard mass at the thyroid and hyperthyroidism secondary to Riedel’s thyroiditis is presented. This case report shows an unusual course with a relapse, until know described in only 4 other published case reports. Riedel’s thyroiditis, also known as Riedel’s struma, is a rare invasive fibrous thyroiditis with a prevalence of 1 per 100,000 people and a male to female ratio of 1/4. Classically, it presents as a stone-like, non-tender thyroid mass with obstructive symptoms such as dyspnea, dysphagia and hoarseness as the structures surrounding the thyroid are involved. Its aetiology is unknown, but it is suggested to be part of a systemic fibrosing disorder. Thyroid dysfunction is possible, mainly hypothyroidism secondary to invasion of the normal thyroid tissue. Ultrasound findings cannot differentiate with anaplastic thyroid carcinoma, a malignancy with an unfavourable prognosis. The diagnosis is based on biopsy findings and the presence of extrathyroidal expansion of the mass. There is no standardized treatment for Riedel’s thyroiditis due to the rarity of the disease. Glucocorticoids are the mainstay of the medical treatment. Surgery is indicated only to relieve compressive symptoms.

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.

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.

Invasive Fibrous Thyroiditis (Riedel Thyroiditis): The Mayo Clinic Experience, 1976–2008

Invasive fibrous thyroiditis (IFT) is the rarest form of thyroiditis, and reports are often limited to case reports and small case series. In this study, we aimed to summarize our institutional experience with IFT since 1976. We retrospectively reviewed the cases of all patients with IFT evaluated at Mayo Clinic, Rochester, Minnesota, from 1976 through 2008, with special emphasis on clinical presentation, associated risk factors, associated comorbid conditions, complications, and treatment. Twenty-one patients met our inclusion criteria of (i) IFT confirmed by pathologic review at our institution and (ii) evidence of extension of fibrosis outside the thyroid capsule. Most patients (17, 81%) were women (mean age, 42 years). Presenting symptoms included pain (24%), dysphagia (33%), vocal cord paralysis (29%), and tracheal narrowing (48%). Three patients had associated hypoparathyroidism. Sixteen (76%) had a history of tobacco use, and 10 (48%) were current smokers. Fibrosing mediastinitis was present in four, orbital fibrosis in one, retroperitoneal fibrosis in three, and pancreatic fibrosis in one (38% had extracervical fibrotic processes). Eighteen patients had partial thyroidectomy, 7 (39%) of whom had surgical complications involving vocal cords and parathyroid. Two required tracheostomy. Thirteen had corticosteroid therapy; six received tamoxifen. There was no cause-specific mortality, and the fibrotic process stabilized or partially resolved in all patients. IFT often is associated with a systemic extracervical fibrotic process and tobacco use. Attempted thyroid resection often results in postoperative complications. Long-term follow-up showed no deaths from IFT and showed stability of the thyroiditis.

Graves’ Disease after Unilateral Riedel’s Thyroiditis

Riedel’s thyroiditis, also termed “invasive fibrous thyroiditis,” is the rarest form of autoimmune thyroiditis and can be associated with systemic fibrotic processes, Hashimoto thyroiditis (1, 2), and Graves’ disease (3). A case of hyperthyroidism followed by spontaneous hypothyroidism and Riedel’s thyroiditis has been reported (4). Two previously reported patients from our institution had invasive fibrous thyroiditis that evolved from antecedent Graves’ disease, as indicated by thyroid dysfunction, bilateral ophthalmopathy, and positive TSH receptor-stimulating autoantibodies (5). We present a new case. In 2004, a 26-yr-old woman presented with a mass in the left thyroid lobe, pain, and respiratory distress. Thyroid function was normal. A tracheostomy and biopsy showed Riedel’s thyroiditis. Therapy with corticosteroids and tamoxifen stabilized the disease. After 1 yr, the tracheostomy was closed. In 2008, her TSH level was less than 0.01 mIU/liter, her free T4 was 4.5 ng/dl (58 pmol/liter; normal, 0.8 –1.8 ng/dl or 10 –23 pmol/ liter), and her serum was strongly positive for TSH receptor antibodies (52%; normal, 16%). The diagnosis of Graves’ hyperthyroidism was made. Her 24-h iodine131 uptake was 33% (upper limit of normal, 29%). She was treated with 19.7 mCi iodine-131. Computed tomography of the neck, performed in 2004 and 2008, showed left lobe thyroid enlargement and compression of the trachea (Fig. 1). Ultrasonography of the thyroid in 2008 (Fig. 2) showed hypervascularity in the areas not affected by Riedel’s thyroiditis; the absence of vascularity in the hypoechoic areas of the left lobe corresponded to Riedel’s involvement, as shown by computed tomography. The present case is of interest because Graves’ disease developed after 3 yr of stability of Riedel’s thyroiditis. In this case, the fibrotic process with extra thyroidal extension was unilateral. Graves’ hyperthyroidism developed in the uninvolved areas of the thyroid. This case and similar reported cases support the notion of autoimmune ISSN Print 0021-972X ISSN Online 1945-7197 Printed in U.S.A. Copyright © 2010 by The Endocrine Society doi: 10.1210/jc.2009-2609 Received December 8, 2009. Accepted April 16, 2010. FIG. 1. Computed tomographic images of the neck. A, Image taken with contrast (2004). Note partial involvement of the left lobe with fibrosis (F), extrathyroidal extension, and significant tracheal narrowing (black arrow) and engulfment of the common carotid artery (white arrow). Also note the uninvolved part of left lobe (L). The right lobe of the thyroid (R) is completely uninvolved. B, Image taken without contrast (2008). Note the stability and partial resolution of the process in the left lobe. (F denotes partially resolved left lobe fibrosis; L, uninvolved part of the left thyroid lobe; R, right lobe. The black arrow shows improved tracheal narrowing.) S P E C I A L F E A T U R E

Soluble intercellular adhesion molecule-1 (sICAM-1) in sera of patients with Graves' ophthalmopathy and thyroid diseases.

Intercellular adhesion molecule, a ligand for the leucocyte integrins CD11a/CD18 (LFA-1) and CD11b/CD18 (Mac-1), that plays an important role in a variety of inflammatory and immune-mediated mechanisms, is strongly expressed in retroocular connective tissue from patients with Graves' ophthalmopathy (GO) and involved in lymphocyte attachment to cultured retroocular fibroblasts via the ICAM-1/LFA-1-mediated pathway. Here, we report the detection and functional activity of a soluble form of the ICAM-1 molecule (sICAM-1) in sera from patients with GO and other thyroid diseases. Serum concentrations for sICAM-1 were determined using a highly sensitive ELISA. Compared with normal controls, patients with hyperthyroid or euthyroid GO and patients with Riedel's invasive fibrous thyroiditis revealed markedly elevated sICAM-1 serum concentrations (all P < 0.0001). In patients with Graves' disease (GD) without clinical GO and in patients with Hashimoto's thyroiditis (HT), sICAM-1 levels were elevated to a lesser degree (both P < 0.001). sICAM-1 serum levels in patients with non-autoimmune hyperthyroidism due to a toxic adenoma were not significantly different from normal controls. In a separate group of 12 patients with severe inflammatory GO, sICAM-1 serum levels markedly declined (P < 0.0001) within 3 months of glucocorticoid therapy in nine patients who responded to this form of treatment with a decrease in periorbital inflammation. In contrast, sICAM-1 serum levels remained unchanged in three patients with poor response to steroids and persistent inflammatory periorbital disease. When tested in a cell adhesion assay, GO sera containing elevated concentrations of sICAM-1 were found to enhance the attachment of peripheral blood mononuclear cells (PBMC) to interferon-gamma (IFN-gamma)-treated retroocular fibroblasts in a dose-dependent manner, up to a maximal stimulation of approximately 5.5-fold (P < 0.001). This effect was abolished by preabsorption of sera with a MoAb against ICAM-1 and inhibited, in a dose-dependent manner, by coincubation with increasing concentrations of purified sICAM-1. In conclusion, sICAM-1 concentrations are markedly elevated in sera from patients with GO, and changes in sICAM-1 serum levels during glucocorticoid therapy closely parallel changes in the degree of inflammation. Given the capacity of sICAM-1 to modulate the adhesion of lymphocytes to retroocular fibroblasts in vitro, sICAM-1 may play a role in the ongoing immune process within the connective tissue in GO.

De Quervain's thyroiditis

Thyroiditis is an inflammation (not necessarily an infection) of the thyroid gland. Inflammatory diseases, are the most common thyroid disorders encountered in clinical practice. Some of these present to the otolaryngologists, as painful deglutition or odynophagia, which can be a very distressing symptom. They may also present as neck pain, sore throat, neck mass or even dyspnoea. Singer classified inflammatory diseases of the thyroid into three broad categories: acute, subacute and chronic thyroiditis. Subacute disease includes granulomatous or De Quervain's thyroiditis and lymphocytic thyroiditis or silent thyroiditis. The chronic group includes chronic lymphocytic (Hashimoto's thyroiditis) and invasive fibrous (Riedel's) thyroiditis [1]. De Quervain's thyroiditis was first described in 1904 and is much less common than Hashimoto's thyroiditis and may be missed if not looked for in a case of odynophagia. The gland swells up and is very painful and tender. As thyroid hormones are discharged into the blood, patient becomes hyperthyroid but the gland cannot take up iodine so the radioactive iodine uptake is very low. The hyperthyroidism generally resolves after a few weeks. The relief of pain by giving steroids in this condition is so dramatic as to be almost diagnostic. Such a case who went from pillar to post for more than 3 weeks, before a diagnosis was made is being reported, to increase awareness of this not so common disorder among clinicians [2].

Tissue eosinophilia and eosinophil degranulation in Riedel's invasive fibrous thyroiditis.

The etiology of Riedel's invasive fibrous thyroiditis (IFT) has remained obscure. This rare disorder has been confused in the past with the more common fibrous variant of Hashimoto's disease. The typical histological features of IFT, in particular the presence of an invasive fibrosclerotic process in conjunction with a prominent chronic inflammatory infiltrate, suggest that the release of fibrogenic cytokines and other factors from these cellular infiltrates may play an important role in the pathogenesis of this condition. Our observations in routinely processed tissue sections obtained from patients with documented IFT of striking tissue eosinophilia led us to hypothesize that eosinophils and their products may play a role in the evolution of this disease. Immunofluorescence staining with affinity-purified polyclonal rabbit antibody directed against human eosinophil granule major basic protein revealed marked tissue eosinophilia and abundant extracellular deposition of major basic protein in all specimens from 16 patients with IFT. By contrast, only occasional eosinophils and no extracellular major basic protein were detected in control thyroid tissues obtained from patients with multinodular goiter, Graves' disease, Hashimoto's disease, and normal thyroid tissue. The presence of marked eosinophil infiltration and extracellular major basic protein deposition in IFT and other associated fibrosclerotic conditions suggests a role for eosinophils and their products in propagating the fibrogenesis seen in IFT.

Tissue eosinophilia and eosinophil degranulation in Riedel's invasive fibrous thyroiditis

Tissue eosinophilia and eosinophil degranulation in Riedel's invasive fibrous thyroiditis

Riedel’s thyroiditis associated with Hashimoto’s thyroiditis, hypoparathyroidism, and retroperitoneal fibrosis

A patient with Riedel's struma (invasive fibrous thyroiditis) with hypothyroidism, hypoparathyroidism, and retroperitoneal fibrosis is reported. This disorder appeared to emanate from Hashimoto's thyroiditis in this case. The possible relationship of the two entities is discussed.

Riedel's Thyroiditis

Invasive fibrous (Riedel's) thyroiditis is a rare thyroid condition of unknown origin that may be associated with inflammatory fibrosclerosing processes elsewhere in the body. Although the condition is benign and self-limiting, its importance lies in its ability to clinically mimic carcinoma almost completely, necessitating performing an open biopsy to establish the correct diagnosis. In a review of over 700 thyroid operations performed at Loma Linda (Calif) University Medical Center in the past 15 years, we encountered only one documented case of Riedel's thyroiditis. Our patient presented with a firm thyroid mass, vocal cord paralysis, and symptoms of esophageal compression. Surgery was performed to obtain a definitive diagnosis and prevent the possibility of subsequent additional tracheal compression with airway compromise. The pathologic findings, as well as the medical and surgical treatment of this condition, are reviewed.

Thyroiditis: A Clinical Update

Thyroiditis may be categorized as acute (suppurative), subacute (granulomatous or lymphocytic), or chronic (invasive fibrous or lymphocytic). Acute suppurative thyroiditis is typically caused by a bacterial infection and resolves with appropriate antibiotic treatment. The subacute thyroiditides are characterized by spontaneously resolving hyperthyroidism associated with low radioiodine uptake, often followed by transient hypothyroidism. Neck pain is the initial symptom in subacute granulomatous thyroiditis, and the disorder recurs only in a minority of patients. Subacute lymphocytic thyroiditis is typically painless, often occurs in the postpartum period, and is being increasingly recognized in the Great Lakes area of the United States. Invasive fibrous thyroiditis (Riedel's struma) is exceedingly rare, often mimics carcinoma, and is associated with extracervical foci of fibrosclerosis. Chronic lymphocytic (Hashimoto's) thyroiditis, an organ-specific autoimmune disease, occurs in at least 2% of women. Although the disorder often produces hypothyroidism, the type of thyroid dysfunction present in patients with Hashimoto's disease reflects the character of the dominant thyroid autoantibody--that is, destructive, blocking, or stimulatory.

The surgical aspects of thyroiditis

Abstract All cases of thyroiditis for the five year period 1957 through 1961 at the Mayo Clinic were reviewed with special reference to the surgical aspects of the disease. It is pointed out that every case of invasive fibrous thyroiditis (Riedel's struma) is a surgical problem. The condition strongly resembles carcinoma on the basis of clinical findings and on gross examination at operation. The diagnosis of granulomatous thyroiditis is usually based on clinical and laboratory findings. Needle biopsy is indicated in the atypical case. In approximately 10 per cent of patients an asymptomatic lump is the only finding, and surgical removal is necessary to exclude carcinoma. In the majority of patients with Hashimoto's disease, needle biopsy is advisable for definitive diagnosis. Failure to respond to medical treatment or the presence of suspicious findings on palpation provides reason for surgical resection.

The surgical aspects of thyroiditis

All cases of thyroiditis for the five year period 1957 through 1961 at the Mayo Clinic were reviewed with special reference to the surgical aspects of the disease. It is pointed out that every case of invasive fibrous thyroiditis (Riedel's struma) is a surgical problem. The condition strongly resembles carcinoma on the basis of clinical findings and on gross examination at operation. The diagnosis of granulomatous thyroiditis is usually based on clinical and laboratory findings. Needle biopsy is indicated in the atypical case. In approximately 10 per cent of patients an asymptomatic lump is the only finding, and surgical removal is necessary to exclude carcinoma. In the majority of patients with Hashimoto's disease, needle biopsy is advisable for definitive diagnosis. Failure to respond to medical treatment or the presence of suspicious findings on palpation provides reason for surgical resection.

Granulomatous thyroiditis (De Quervain's thyroiditis).

GRANULOMATOUS thyroiditis is a distinct clinicopathologic entity, apparently a true nonsuppurative inflammation of the thyroid gland, that can be distinguished from the rare invasive fibrous thyroiditis (Riedel's struma) or the more common struma lymphomatosa. Since its first description by de Quervain in 1904 (1), the disease has been reported under many synonyms. Terms that call attention to the histologic features of the lesion include “pseudotuberculous thyroiditis,” “granulomatous thyroiditis,” “giant-cell thyroiditis” or “struma fibrosa, giant-cell variant.” The clinical findings have given origin to such names as “acute and subacute nonsuppurative thyroiditis,” “acute noninfectious thyroiditis,” “acute diffuse thyroiditis” and “subacute thyroiditis.” The latter term is commonly used. However, since there appears to be one basic pathologic process with extreme variation in severity of symptoms, and since many cases undoubtedly occur in which subjective symptoms are absent, it was thought best to use...

Invasive fibrous thyroiditis (Riedel's struma).

INVASIVE fibrous thyroiditis as herein defined is a rare clinicopathologic entity. The exact incidence cannot be ascertained readily from the literature, largely because many authors have not defined accurately the type of “thyroiditis” being reported. Thus, critical appraisal of all purported cases is necessary. Prior to 1932, it was widely held that Riedel's struma was an end stage of Hashimoto's disease. Graham (1), in a careful review of the literature up to 1929, emphasized the striking differences between the two diseases and considered only 41 cases of Riedel's struma 2) reported up to that time as authentic. Since Graham's review, surprisingly few additional cases have been reported (3). A considerable number of reports on Riedel's struma deal in part or entirely with granulomatous (subacute) thyroiditis (4–11). The number of cases in which a strict definition of invasive fibrous thyroiditis is adhered to is extremely small. Schilling (12) described 1 authentic case with necropsy findings and 1 ad...