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vol. 15
State of the art paper

IgG4-related disease in endocrine practice

Agata Rzepecka, Anna Babińska, Krzysztof Sworczak

Arch Med Sci 2019; 15, 1: 55–64
Online publish date: 2017/10/20
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IgG4-related disease is a set of symptoms resulting from a chronic, usually multiple organ inflammatory condition which affects various organs. It consists of lymphoplasmacytic infiltrations with attendant fibrosis and deep vein thrombosis. Frequently observed tissue lesions are accompanied by elevated IgG4 levels in serum. The etiopathogenesis of the lesions is of multifactor character and the clinical manifestation of the disease is highly diverse. The diagnostic process is based on the patient’s medical history, clinical examination and additional tests, including a histopathological examination of the infected organ’s tissues. Almost forty different locations of the disease have been reported, including disorders of the endocrine system. IgG4-related endocrinopathies are quite rare. However, it is likely that the diagnosis is under-reported due to lack of awareness of this clinical entity. Despite increasing interest in the subject, there are not enough reliable studies evaluating the link between IgG4-RD and endocrine disorders.

IgG4-related disease, Hashimoto’s thyroiditis, Riedel’s thyroiditis, hypophysitis, Graves’ ophthalmopathy

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