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2016 ; 5
(4
): 231-239
Nephropedia Template TP
Eur Thyroid J
2016[Dec]; 5
(4
): 231-239
PMID28101487
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Immunoglobulin G4-related disease (IgG4-RD) is a new disease category involving
many organ systems, including the endocrine system in general and the thyroid in
particular. Since an initial association was made between hypothyroidism and
autoimmune (IgG4-related) pancreatitis, more forms of IgG4-related thyroid
disease (IgG4-RTD) have been recognized. Four subcategories of IgG4-RTD have so
far been identified: Riedel thyroiditis (RT), fibrosing variant of Hashimoto
thyroiditis (FVHT), IgG4-related Hashimoto thyroiditis, and Graves disease with
elevated IgG4 levels. Although a male predominance is seen for IgG4-RD in
general, RT and FVHT have a female preponderance. The pathogenesis of IgG4-RD is
not completely understood; however, genetic factors, antigen-antibody reactions,
and an allergic phenomenon have been described. Diagnosis of IgG4-RD requires a
combination of clinical features, serological evidence, and histological
features. Histology is the mainstay of diagnosis, with IgG4 immunostaining.
Although serum IgG4 levels are usually elevated in IgG4-RD, raised serum IgG4 is
neither necessary nor adequate for diagnosis. Imaging supports the diagnosis and
is a useful tool in disease monitoring. Management of IgG4-RTD is both medical
and surgical. Steroids are the first-line treatment and may produce a swift
response. Tamoxifen and rituximab are second-line agents used in
steroid-resistant patients. Surgical debulking is carried out in RT solely as a
procedure to relieve obstruction. Other endocrine associations described with
IgG4-RD are hypophysitis and Hashimoto encephalopathy. IgG4-RTD is an uncommon
disease entity, and prompt diagnosis and treatment can improve outcomes.