Riedel's thyroiditis, is a chronic form of

thyroiditis Thyroiditis is the inflammation of the thyroid gland. The thyroid gland is located on the front of the neck below the laryngeal prominence, and makes hormones that control metabolism. Signs and symptoms There are many different signs and sympto ...

. It is now believed that Riedel's thyroiditis is one manifestation of a systemic disease that can affect many organ systems called

IgG4-related disease IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and ...

. It is often a multi-organ disease affecting pancreas, liver, kidney, salivary and orbital tissues and retroperitoneum. The hallmarks of the disease are fibrosis and infiltration by IgG4 secreting plasma cells.

Signs and symptoms

Pathophysiology

Riedel's thyroiditis is characterized by a replacement of the normal thyroid parenchyma by a dense

fibrosis Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perma ...

that invades adjacent structures of the neck and extends beyond the thyroid capsule. This makes the thyroid gland stone-hard (woody) and fixed to adjacent structures. The inflammatory process infiltrates muscles and causes symptoms of tracheal compression. Surgical treatment is required to relieve tracheal or esophageal obstruction.

Diagnosis

It typically present as a painless, hard anterior neck mass, which progresses over weeks to years to produce symptoms of compression, including dysphagia, dyspnea choking and hoarseness. Patients may present with symptoms of hypothyroidism and hypoparathyroidism as the gland is replaced by Fibrous tissues .physical examination reveals a hard,"woody" thyroid gland with fixation to surrounding tissue. The diagnosis needs to be confirmed by open thyroid biopsy because the firm and Fibrous nature of the gland renders FNAB inadequate

Treatment

Therapy usually consists of

prednisolone Prednisolone is a steroid medication used to treat certain types of allergies, inflammatory conditions, autoimmune disorders, and cancers. Some of these conditions include adrenocortical insufficiency, high blood calcium, rheumatoid arth ...

, nonetheless some cases may require surgery.

Tamoxifen Tamoxifen, sold under the brand name Nolvadex among others, is a selective estrogen receptor modulator used to prevent breast cancer in women and treat breast cancer in women and men. It is also being studied for other types of cancer. It has b ...

has been proposed as part of a treatment plan. Treatment is directed to surgical relief of compressive symptoms. Tamoxifen may also be beneficial. The type of surgery which is indicated here is isthmectomy.

Prevalence

Riedel's thyroiditis is classified as rare. Most patients remain

euthyroid The thyroid, or thyroid gland, is an endocrine gland in vertebrates. In humans it is in the neck and consists of two connected lobes. The lower two thirds of the lobes are connected by a thin band of tissue called the thyroid isthmus. The thy ...

, but approximately 30% of patients become

hypothyroid Hypothyroidism (also called ''underactive thyroid'', ''low thyroid'' or ''hypothyreosis'') is a disorder of the endocrine system in which the thyroid gland does not produce enough thyroid hormone. It can cause a number of symptoms, such as po ...

and very few patients are hyperthyroid. It is most commonly seen in women.

Eponym

It is named for Bernhard Riedel. He first recognized the disease In 1883 and published its description in 1896.

References

External links

{{Thyroid disease IgG4-related disease Steroid-responsive inflammatory conditions Thyroid disease