Amyloid goiter as a manifestation of primary systemic amyloidosis

Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Thyroid (Impact Factor: 3.84). 02/2007; 17(1):77-80. DOI: 10.1089/thy.2006.0045
Source: PubMed

ABSTRACT Primary amyloidosis involving the thyroid gland is rare and limited to case reports. We report the case of a previously healthy 47-year-old female presenting with a 1-month history of nausea, vomiting, and diffuse thyroid enlargement. Over the next 3 months, she simultaneously developed renal insufficiency dysphagia and hoarseness of voice. Biopsies from the gastric antrum, duodenum, bone marrow, and kidney were positive for vascular deposition of amyloid. Ultrasound of the thyroid revealed diffuse enlargement of the thyroid gland, which was 32.8 ML in volume, with diffuse hyperechogenicity. Fine needle aspiration (FNA) biopsy was positive for amyloid by Congo red staining, and cytology was negative for malignancy. The patient was treated with dexamethasone 40 mg daily on days 1-4, 9-12, and 17-20 for 3 months. On 3-month follow-up, the patient's nausea and vomiting had resolved and renal insufficiency improved. Ultrasound of the thyroid demonstrated decrease in the size of the goiter to 23.2 ML. Amyloid goiter is seen only in approximately 0.04% of patients with primary systemic amyloidosis. No data is currently available regarding treatment of primary amyloidosis and its effect on the goiter. However, we have evidence demonstrating that successful treatment of amyloidosis decreases thyroid enlargement and improves organ dysfunction.

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    05/2011; 3:97-99. DOI:10.5005/jp-journals-10002-1067
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    ABSTRACT: Amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. The most common forms of systemic amyloidosis are primary amyloidosis (PA) of light chains and secondary amyloidosis (SA) caused by chronic inflammatory diseases such as rheumatoid arthritis (RA). Although involvement of the thyroid gland by amyloid is a relatively common phenomenon, clinically significant enlargement of the thyroid owing to amyloid deposition is a rare occurrence. In SA, the deposition of amyloid associated (AA) protein is associated with atrophy of thyroid follicles. The clinical picture of these patients is characterized by rapid, painless thyroid gland enlargement which may be associated with dysphagia, dyspnea, or hoarseness. Thyroid function is not impaired in most cases. Although amyloid goitre secondary to systemic amyloidosis due to chronic inflammatory diseases is relatively common, specifically related to RA is much more uncommon one and it is reported less in the literature. In this report, A 52-old-year female patient with amyloid goiter associated with amyloidosis secondary to rheumatoid arthritis is presented.
    Case Reports in Medicine 12/2013; 2013:792413. DOI:10.1155/2013/792413
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    ABSTRACT: Introduction Amyloid goiter (AG) is characterized by enlargement of the thyroid gland as a result of extensive amyloid deposition in a bilateral and diffuse manner. Presentation of Case 58 year-old male patient was diagnosed of Crohn's Disease (CD). He was admitted to our clinic with complaint of respiratory distress and rapid growth swelling in the neck. Ultrasound examination revealed huge multinodular goiter on both sides of thyroid gland. We performed bilateral total thyroidectomy. Pathological evaluation revealed AG. Discussion Amyloid leads to degeneration in tissues, thereby disrupts the function of the relevant organs. It is important to distinguish AG from other reasons of goiter, particularly thyroid medullary cancer that can cause amyloid deposition in thyroid gland. Secondary amyloidosis frequently involves thyroid gland at microscopic level, but rarely causes goiter. An analysis of current literature revealed that only few cases of AG occurred secondary to CD. Herein we presented a case of AG who has rapidly growing goiter that associated with CD. Conclusion AG must be kept in mind in case of rapidly growing goiter, especially in patients with chronic inflammatory bowel diseases.
    06/2014; 5(8). DOI:10.1016/j.ijscr.2014.06.004