Riedel’s thyroiditis: clinical presentation, treatment and outcomes

Abstract

BackgroundRiedel´s thyroiditis (RT) is a rare inflammatory disease of the thyroid gland, causing compression and fibrosis of adjacent tissues. Typically the goiter is hard and firm. Hoarseness, dyspnea, and dysphagia may be present.MethodsWe retrospectively reviewed all patients known by us with RT in addition to all patients with appropriate ICD-10 codes evaluated at the Karolinska University Hospital 2003–2015. Clinical, biochemical, and histological data of patients with RT were recorded in detail. Histological preparations were re-examined when available.ResultsRT was diagnosed in six patients. Five were females and the median age at first presentation was 50 years (25–81 years). Median follow-up time was 3.75 years (1–22 years). At diagnosis five had hypothyroidism. Four had extrathyroidal manifestations, and one of these had also distant fibrosis. One patient had a clear IgG4/IgG ratio over 40%. One patient was treated with tracheostomy, one with isthmectomy and one with total thyroidectomy. Four had been treated with glucocorticoids, four with tamoxifen, and two with both drugs. One had also been treated with mycophenolate mofetil combined with Rituximab. At the end of follow-up four was doing fine, one had recurrent episodes of inflammation and one had died of possible complications to RT.ConclusionIt is important to recognize RT and give adequate treatment. Steroids are still the mainstay of therapy but other medications against fibrosclerosis can be considered. Wakefulness of other fibrosing manifestations is essential. Immunohistochemistry can show whether IgG-4 plasma cells are increased which could lead to fibrosis in other organs.

Difficulties in diagnosis of Riedel's thyroiditis on aspiration cytology: A case report and brief review of the literature

“…Fibrosing variant of HT differs from RT in the absence of extrathyroidal extension of fibrosis and frequent presence of germinal center cells and oncocytic cells in the FNA smears. Raised antithyroid antibodies also help in this distinction, though few cases of RT may also have high antimicrosomal antibody titers . Subacute or deQuervain's thyroiditis presents clinically with a tender enlargement of thyroid accompanied by fever.…”

“…10,11,18,22,23 The cytological differential diagnosis of RT can include benign also help in this distinction, though few cases of RT may also have high antimicrosomal antibody titers. 6,11,18 Subacute or deQuervain's thyroiditis presents clinically with a tender enlargement of thyroid accompanied by fever. FNA smears typically demonstrate numerous giant cells, epithelioid cell granulomas and degenerating follicular cells.…”

Hypothyroidism

“…Riedel's thyroiditis is a fibrosclerosing thyroiditis of unknown etiology, with a probable primary anti-immune or fibrotic origin similar to retroperitoneal fibrosis, fibrosing mediastinitis, sclerosing cholangitis, and lacrimal fibrosis, among others [43,44].…”

“…From 30 to 60% of patients present clinical or subclinical hypothyroidism. It is one of the IgG4-related disease varieties, together with Fibrosing hashimoto thyroiditis, Igg4related Hashimoto's disease, and Graves' disease associated with IgG4 [43][44][45].…”

Riedel's Thyroiditis. A Clinical Review.

“…Тиреоидит Риделя (fibrosing thyroiditis, Riedel's thyroiditis) является чрезвычайно редкой формой тиреоидита, характеризующейся замещением паренхимы щитовидной железы соединительной тканью. Наиболее частыми клиническими проявлениями являются «деревянистая» плотность железы, несмещаемость при пальпации, сдавление соседних структур (трахея, пищевод, сосуды и нервы) [1,2]. Это заболевание названо в честь немецкого хирурга Риделя, который сообщил о нескольких случаях тиреоидита с выраженным фиброзом на международном конгрессе хирургии в 1894 году.…”