IGG4-related diseases in endocrinology

Abstract

Immunoglobulin-G4-related disease (IgG4-RD) is a chronic immunomediated pathology of different organs of local or systemic nature, which has been established as a separate clinical entity in the early 2000s and is characterized by storiform fibroid inflammation of the affected tissues, their increase, and elevated serum immunoglobulin-G4 (IgG4) levels. The most common manifestations of the disease are major salivary and lacrimal gland enlargement, lymphadenopathy and type 1 autoimmune pancreatitis (AIP1), however, other organs may be also involved (the thyroid, eyes, meninges, heart, lungs, kidneys, aorta, upper airways, mesentery, etc.).
 The effectiveness of treatment of IgG4-RD, as well as other pathological conditions, is also determined by the timely diagnosis. However, the latter is complicated due to the variety of clinical manifestations and rather variable diagnostic criteria. It is necessary to constantly update the evidence-based knowledge and diagnostic algorithms within this pathology in order to overcome the difficulties, and involve immunologists, endocrinologists, pathologists and specialists in other spheres.
 This review provides information about the etiology, pathogenesis, and current methods of diagnosis and treatment of IgG4-related diseases, as well as examples of some manifestations of IgG4-RD that an endocrinologist may face in practice.

Reshaping the Concept of Riedel’s Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease)

“…IgG4-mediated endocrine diseases are IgG4-related thyroiditis of four types, as mentioned above, and IgG4-associated hypophysitis (which has been clarified within the last decade, but it is still a matter of debate in certain areas) [ 101 , 102 , 103 , 104 ]. Generally, head and neck spreading of IgG4-RD involve the two endocrine glands, thyroid, and pituitary gland, in addition to lacrimal, and salivary glands, orbit, as well as meningeal area [ 105 ].…”

The clinical case of IgG4-related thyroid disease in a 6-year-old child

“…и соавт. опубликован литературный обзор, включивший в себя вопросы этиологии, классификации, иммунопатогенеза, клинической картины, диагностики и лечения IgG4-связанных заболеваний [ 13 ].…”

“…У 60–90% пациентов наблюдается поражение двух и более органов [ 14 ]. Заболевание может манифестировать одновременно в нескольких органах или постепенно, вовлекая один орган за другим, что требует тщательного наблюдения за пациентом [ 13 ].…”

“…Преимущественно клинические проявления IgG4-связанного заболевания носят подострый характер из-за прогрессирующего увеличения органов, и в ряде случаев заболевание выявляется случайно во время диагностических исследований. В 40% случаев у пациентов с IgG4-связанным заболеванием имеется отягощенный аллергологический анамнез (бронхиальная астма, аллергический ринит, атопический дерматит) [ 13 ][ 14 ].…”