Introduction and objectives:

IgG4-Related disease (IgG4-RD) is a systemic autoimmune disorder characterized by tissue infiltration with IgG4-secreting plasma cells, fibrosis and organic disfunction. It can involve multiple sites. The objectives in this article are to identify the laryngeal involvement of this disease.

Methods:

A systematic review was conducted by collecting the literature published between 2003 and February 2021, seeking for case reports of IgG4-RD with laryngeal involvement. The bibliographic databases utilized were Pubmed, Web of Science and Scopus. 22 cases were selected, all of them including histological findings in the larynx.

Results:

Most of the patients were male, between 41 and 60 years old. The most common laryngeal manifestations were pseudotumoral neoformations. The supraglottis was the area were most of these lesions appeared. In this disease the standard treatment involves glucocorticoids, often with an optimal clinical response. However, a great part of the patients included in the review got a delayed diagnosis, some of them leading to aggressive surgical procedures.

Conclusions:

Laryngeal manifestations in the IgG4-Related disease are unusual. That's why it should be considered when considering the differential diagnosis of malignant tumors. This is an illness that needs to be suspected in some cases, to ensure a prompt diagnosis and a proper treatment, thus avoiding long-term sequelae.

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