History Of Hashimoto's Thyroiditis Research Articles

7658 A Case of Recurrent Cardiac Tamponade as Initial Presentation of Primary Adrenal Insufficiency

Abstract Disclosure: A. Farooq: None. A. Ghumatkar: None. J. Snitzer: None. Introduction:Cardiac tamponade, a life-threatening emergency, can be associated with primary adrenal insufficiency (Addison Disease), and accurate diagnosis requires a high degree of clinical suspicion in patients with uncertain etiology and nonspecific signs and symptoms. Case Presentation:A 58-year-old female with a history of Hashimoto thyroiditis was evaluated in the hospital for two episodes of cardiac tamponade requiring emergent pericardiocentesis within one month. The patient had been experiencing symptoms of fatigue, nausea, lightheadedness, dizziness, hiccups, and tanned skin for the past year. She also had a recent flu-like illness with nausea and vomiting four months before her current presentation. Her vitals were notable for hypotension during these episodes. Her lab work showed hyponatremia (128 mEq/L, reference range: 136-145 mEq/L) with normal potassium and glucose levels. Her thyroid function tests were within normal limits on her home levothyroxine dosage (TSH 4.588 IU/ml, reference range: 0.5-8.9 IU/ml). An ACTH stimulation test was performed based on the patient's clinical presentation, consistent with adrenal insufficiency. Her baseline cortisol level was 5.9 mcg/dL (reference range: 6.7-22 mcg/dL), a post-ACTH stimulation level of 6.7 mcg/dL after 30 minutes, and 6.0 mcg/dL after 60 minutes. Her 21 alpha-hydroxylase antibody titer was also positive for Addison Disease (231U/mL, reference range <1 IU/mL). A CT scan of the abdomen with intravenous contrast was done, which revealed no abnormality of the adrenal glands. She was treated with stress doses of hydrocortisone during hospitalization and discharged with maintenance dosage and follow-up with endocrinology. She had another episode of acute adrenal crisis about a month later but had no pericardial effusion this time and responded well to the treatment with stress doses of steroids. She has remained healthy at 14 years of follow-up. Conclusion: This case emphasizes the need to consider adrenal insufficiency in the evaluation of cardiac tamponade or pericardial effusions, especially in patients with existing autoimmune glandular disorders. This patient may have autoimmune polyglandular syndrome type 2 (APS-2), which is a cluster of two or more endocrine glandular disorders primarily diagnosed in adulthood and predominant in females. The risk of adrenal crisis is more than twofold in Addison disease due to APS-2. Lastly, this case highlights that hiccups can be related to adrenal insufficiency, with pathophysiology possibly involving diaphragmatic irritation due to adrenal inflammation that resolves with treatment. Presentation: 6/3/2024

FRI531 IgG4 Related Mass -forming Thyroid Disease: Two Case Reports

Abstract Disclosure: R.S. Braham: None. C. Droumaguet: None. M. Vitellius: None. J. Tran-van-nhieu: None. E. Béquignon: None. P. Desgranges: None. N. Limal: None. M. Mahevas: None. B. Godeau: None. IgG4 thyroiditis is an invasive fibrotic process involving the thyroid in a systemic disease. It is a rare inflammatory autoimmune disease with insidious presentation and nonspecific symptoms.We describe two unusual cases of mass forming IgG4 thyroiditis that were diagnosed recently in our department. The first case is about a 42-year-old woman who has a history of Hashimoto thyroiditis with hypothyroidism presenting with a rapid goiter growth, neck pain, dysphagia and hoarseness. A lymphoma was suspected. Ultrasonography shows heterogeneous hypoechoic lesions that may infiltrate the perithyroid muscles. Fine-needle aspiration (FNA) was suspicious of IgG4 infiltrative disease which was confirmed by microbiopsy. Only the thyroid was hypermetabolic on fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) Scan. No improvement under corticosteroid treatment and Rituximab. Surgery was planified to relieve pressure symptoms. The thyroid surgery was very difficult because of the extensive fibrosis and only a biopsy was performed, confirming the diagnosis and excluding malignancy. The second case is about a 68-year-old woman with a history of hypertension and Graves disease presented with a neck right mass that was suspected to be a paraganglioma, hypermetabolic on FDG PET scan with no other localisation. This mass was surrounding the right primitive carotid artery, associated with thrombi of the internal jugular vein. FNA and microbiopsy found a fibrotic non specific tissue. Thyroidectomy and surgical biopsy of the neck mass were performed with histopathology confirming IgG4 disease on the thyroid gland with fibrosis. There were no hypocalcemia in the two cases and no other localisation. IgG4 related mass-forming thyroiditis is an extremely rare disease. It is now a recognizable pattern of IgG4 thyroiditis, and has as differential diagnosis Hashimoto’s thyroiditis, Graves disease and thyroidal carcinoma.We will discuss through these cases the different clinical presentation of this rare disease, the diagnostic tools, the treatment and the long term follow up. Presentation: Friday, June 16, 2023

Adrenal hemorrhage following direct oral anticoagulant (DOAC) therapy: two case reports and literature review

BackgroundAdrenal hemorrhage (AH) is a rare condition that can result in a life-threatening medical emergency. This medical condition could be caused by several underlying factors, one of which is the use of anticoagulants. As far as we are aware, direct oral anticoagulant (DOAC) agents are a rare but possible cause of AH.Case presentationHerein, we described two cases of AH due to DOACs. The first case was a 35-year-old Iranian woman with a past medical history of Hashimoto thyroiditis who was being treated with apixaban due to the previous thrombosis. Her first symptoms of AH (November 2021) were strangely similar to symptoms of autoimmune Addison disease (AAD) which led to a confirmed diagnosis of autoimmune polyendocrine syndrome type 2 (APS-2). An abdominal MRI revealed an oval shape well-encapsulated cystic mass with a diameter of 20 × 14 mm with a thick and low signal intensity rim in the left adrenal gland, highly suggestive of sub-acute left-sided AH. Our second case was an 89-year-old Iranian woman who had been admitted to the hospital (August 2021) with low blood pressure and disorientation. At the beginning of her admission, the evaluation showed hyponatremia, and further evaluations confirmed adrenal insufficiency (AI). The patient reported rivaroxaban usage for deep vein thrombosis prophylaxis after femur fixation surgery. Her abdominal CT scans showed bilateral adrenal masses highly suggestive of AH. Her follow-up examination showed persistent AI after three months.ConclusionGiven the history of our cases, physicians should be aware of AH in patients receiving DOACs, particularly in elderly patients who are at high risk of bleeding. It is also worth noting that AH can occur in any patient with any medical history and history of DOAC use, which is why patients must be closely monitored.

Primary thyroid lymphoma

Primary thyroid lymphoma represents a challenging, multi-disciplinary condition that it may be found in addition to positive antibodies against thyroid. Early recognition is essential for an adequate management. A thyroid nodule but usually the entire goiter may rapidly progress to a voluminous mass causing local compressive symptoms. Most of the patients with primary thyroid lymphoma had a history of Hashimoto thyroiditis. Epidemiologically, primary thyroid lymphoma represents an exceptional entities opposite to papillary or follicular cancer. The first tool of evaluation is ultrasound; in order to appreciate the local and distance invasion, computed tomography is useful. Fine needle aspiration and cell block analysis or biopsy and immunohistochemistry report are essential for adequate diagnostic in order to avoid unnecessary surgery. Diffuse large B-cell lymphoma is the most frequent histological type. Chemotherapy and local radiation represents the elective management. Considering this unusual condition, awareness is the key operative word for different practitioners.

Clinical Images: Spotted bone: Incidental detection of a rare genetic disease.

The patient, a 27-year-old woman with a history of Hashimoto thyroiditis, presented to the rheumatology clinic for upper and lower extremity swelling. A physical examination demonstrated hand swelling and flesh-colored lichenified papules on her sacrum, neck, and ankles. Results of laboratory workup showed weakly positive antinuclear antibodies (1:320) with a nuclear fine-speckled pattern but otherwise no autoantibodies. A skin biopsy specimen demonstrated thickened dermal collagen consistent with connective tissue nevus. We obtained anterior–posterior (AP), axial, and Grashey views on radiographs of both shoulders (A); AP, lateral, and oblique radiographs of both feet (B); and posterior–anterior, lateral, and oblique radiographs of both hands (C). Each view demonstrated diffusely distributed small sclerotic densities overlying the normal osseous structures consistent with scattered enostoses. The differential diagnosis included sclerosing bony dysplasias (sporadic or inherited), sclerotic metastases, osteomas, and osteosarcomas (1). Genetic testing revealed a heterozygous mutation in LEMD3. Her constellation of findings was consistent with Buschke-Ollendorff syndrome, an autosomal dominant condition in which diagnostic features include osteopoikilosis, connective tissue nevi, an LEMD3 mutation, and a family history (2). Loss-of-function mutations in LEMD3, a gene involved in bone morphogenic protein signaling, have been previously described in cases of Buschke-Ollendorff syndrome, although the exact relationship is yet unknown (3). Imaging features that may help differentiate osteopoikilosis from other malignant disorders include its preponderance for the epiphyseal regions of short tubular bones, lesion size less than 1 cm, homogenously radiodense lesions with spiculated margins, and lack of uptake on bone scintigraphy (1, 4). Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Primary Thyroid Diffuse Large B-cell Lymphoma in a Child with Hashimoto’s Thyroiditis: A Case Report

Primary thyroid lymphoma (PTL) is a rare thyroid gland cancer, with diffuse large B-cell lymphomas (DLBCL) being extremely rare in children and adolescents. Thus, optimal therapy is debatable. We describe a rare case of thyroid DLBCL in an adolescent girl with a history of Hashimoto thyroiditis (HT), the difficulty in diagnosis and the outcome of treatment. A 12-year-old girl with a nine-year history of HT was admitted with a right-sided painless progressive swelling of the neck. Physical examination and imaging including ultrasound (US), computed tomography (CT) and positron emission tomography/CT revealed an enlarged thyroid gland with right side lymphadenopathy and no metastasis. Two fine needle aspirations were done showing suspected lymphoblastic lesions for non-Hodgkin lymphoma without precise diagnosis. US guided core needle biopsy was finally performed confirming the diagnosis of DLBCL. She was treated according to LMB 96-group B protocol with no surgical removal of thyroid. The patient responded very well to treatment and 14 months later there is no evidence of relapse or metastases. PTL is an extremely rare cause of thyroid malignancy in children. However, it should be considered in the differential diagnosis of a thyroid mass in adolescents presenting with a rapidly enlarging neck mass and a history of HT. It is a treatable condition with a good prognosis, even in aggressive histological subtypes, with no need for thyroidectomy.

Clinical case discussion: Autoimmune polyglandular syndrome type III B+ C in the elderly

This report presents an 84 year-old patient admitted into our hospital for dizziness with a history of Hashimoto's thyroiditis, chronic gastritis and vitiligo.Physical examinations showed skin depigmentation.Laboratory tests revealed anemia, positive intrinsic factor antibodies and normal adrenal function.This patient was finally diagnosed with autoimmune polyglandular syndromes(APS)type Ⅲ B+ C.Recurrent anemia was mainly attributed to malabsorption caused by autoimmune gastritis.This article reviews the common clinical manifestations of APS, in order to call for geriatricians' attention to APS.APS Ⅲ B+ C should be considered if vitiligo combined with anemia occurs in elderly patients. Key words: Autoimmune diseases; Anemia; Vitiligo; Gastritis

Synchronous Occurrence of Papillary Thyroid Carcinoma and Mucosa-Associated Lymphoid Tissue Lymphoma: a Single Case Report

Papillary thyroid carcinoma (PTC) is a common lesion, accounting for 70–80% of all thyroid cancers, whereas mucosa-associated lymphoid tissue (MALT) lymphoma of thyroid gland is rare. A simultaneous occurrence of both malignancies is extremely rare. 57 years old Korean woman diagnosed with Hashimoto' s thyroiditis at left lobe of thyroid gland where atypical cells of undetermined significance at right lobe. Later, left lobe was confirmed with malignant lymphoma during series of fine-needle aspiration biopsy. Right lobe was interpreted as malignant lesions, such as papillary thyroid carcinoma based on ultrasonography images and previous biopsy results. Total thyroidectomy was performed. Pathology reported papillary thyroid carcinoma at right lobe and MALT lymphoma at left lobe. There were no post-operative complications and no recurrence yet reported. Since an association between Hashimoto' s thyroiditis and development of MALT lymphoma has been reported previously, a history of Hashimoto thyroiditis should be suspected MALT lymphoma.

High Incidence of Gastrointestinal Tract Disorders and Autoimmunity in Primary Cutaneous Marginal Zone B-Cell Lymphomas

To our knowledge, this is the first comprehensive study addressing comorbidities associated with primary cutaneous marginal zone B-cell lymphomas (PCMZLs). To determine if patients with PCMZL were at risk for other medical conditions. Case-control study at a cutaneous lymphoma clinic and a dermatopathologic consultation service at a single academic institution using an extensive questionnaire of illnesses, symptoms, and environmental exposures for 80 sequential patients with PCMZL and 80 matched controls. The frequency of several morbidities was obtained in both groups from data gathered from the questionnaire and corroborated by reviewing medical records when available. We found a high incidence of past or present gastrointestinal tract problems in 49 patients (61.2%) with PCMZL compared with 30 participants (37.5%) in the control group (CG) (P = .003). Gastroesophageal reflux was reported in 50.0% (40 vs 27 in the CG, P = .04) and gastric ulcers in 10.0% (8 vs 3 in the CG, P = .13); 20.0% of the cohort had positive Helicobacter pylori serology (16 vs 2 in the CG, P = .003). Colon disorders, including irritable bowel syndrome and inflammatory bowel disease, were more common in the PCMZL cohort (20 vs 7 in the CG, P = .01). Autoimmunity was reported in 20.0% of participants (16 vs 6 in the CG, P = .03). Eight patients had a history of Hashimoto thyroiditis. Three patients had a positive antinuclear antibody. Two had a diagnosis of lupus erythematosus and 1 had Sjögren syndrome. Sicca syndrome was noted in 12.5% (10 vs 3 in the CG, P = .052). A history of noncutaneous malignant neoplasms was observed in 21.3% of the patients (17 vs 8 in the CG, P = .050). Other notable morbidities did not reach statistical significance. Our results indicate a high incidence of systemic conditions in patients with PCMZL, especially involving the gastrointestinal tract, but also autoimmunity and cancer.

The Role of PTPN22 C1858T Gene Polymorphism in Diabetes Mellitus Type 1: First Evaluation in Greek Children and Adolescents

Type 1 diabetes mellitus (T1DM) is an autoimmune multifactorial disease. Protein tyrosine phosphatase nonreceptor type 22 (PTPN22) gene encodes lymphoid-specific tyrosine phosphatase (Lyp), an inhibitor of T cell activation. PTPN22 C1858T polymorphism was associated with T1DM in populations of Caucasian origin. The aim of this study was the investigation for the first time of the association of PTPN22 C1858T polymorphism with T1DM in Greek population. We studied 130 children and adolescents with T1DM and 135 healthy individuals of Greek origin. The polymorphism was genotyped using polymerase chain reaction with restriction fragment length polymorphism. C1858T and T1858T genotypes as well as 1858T allele were found more frequently in patients (10.8% and 5.8%, resp.) than in healthy individuals (5.9% and 3.0%, resp.) but at non statistically significant level. There was no statistically significant association found with gender, age at diagnosis, severity of onset, history of Hashimoto thyroiditis or family history of T1DM. Increased frequency of 1858T allele in patients than in controls, implying a probable association, agrees with results of similar studies on other populations. The inability to find a statistically significant difference is probably due to the decreased frequency of minor allele in Greek population, indicating the need for a larger sample.

Primary Thyroid Lymphoma

Eight cases of primary thyroid lymphoma were reported in a tertiary government hospital from January 2005 to August 2011. All patients presented with a diffuse enlargement of both thyroid lobes with associated obstructive symptoms. Five of these cases were extranodal marginal zone lymphoma and three were diffuse large B-cell lymphoma. Clinical features that would favor a thyroid lymphoma include tumor size of greater that 7cm, obstructive symptoms, clinical hypothyroidism or history of Hashimoto thyroiditis. Thus, these features must be considered in evaluating thyroid nodules during fine-needle aspiration biopsy. Histologically, extranodal marginal zone B-cell lymphoma shows vaguely nodular to diffuse infiltrates of small to intermediate size atypical lymphoid cells infiltrating the thyroid follicles, while diffuse large B-cell lymphoma shows sheets of large atypical lymphoid cells infiltrating the thyroid follicular epithelium.
 Keywords: Primary Thyroid Lymphoma, Extranodal Marginal Zone B-cell Lymphoma, Diffuse Large B-cell Lymphoma.