Spectrum of manifestations of IgG4-related disease: a literature review

Abstract

IgG4-Related Disease is a plasma cell lymphoproliferative condition, associated with increased levels of IgG4 and infiltration of IgG4-positive cells in affected structures. Due to its broad spectrum of manifestations, there are still difficulties in its diagnosis. This study aims to highlight the main clinical manifestations that comprise its spectrum. It is an integrative, descriptive literature review with a qualitative approach, conducted in the databases Embase, PubMed, SCIELO, and Web of Science. Thirty-eight articles were included in this review. It is observed that IgG4-Related Disease has a variable clinical presentation, with reports in the literature of involvement in all organs and systems of the body. The most affected organs are the pancreas, ocular orbits, salivary, and lacrimal glands. It is noteworthy that this variety of manifestations results in different clinical repercussions, which can lead to complicated conditions due to the possibility of affecting vital organs.