Is IgG4-Related Disease a Systemic Autoimmune Disease?

Dear Colleagues,

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by elevated serum IgG4 concentrations, tissue infiltration by IgG4-positive cells, and storiform fibrosis in various organs, including the pancreas, salivary and lacrimal glands, kidneys, retroperitoneum, etc. In particular, those findings regarding IgG4 reported in the early 21st century from Japan provided an important basis for establishing IgG4-RD as a new disease concept. The characteristics of IgG4-RD, such as hypergammaglobulinemia, synchronous or metachronous clinical course, and prompt responsiveness to glucocorticoids, strongly suggest that an autoimmune mechanism exists in the pathophysiology of this disease. In fact, several autoantibodies have recently been reported from some facilities. Considering the pathogenic role of T cells, T helper type 2 cells’ and regulatory T cells’ dominant milieu in the blood and lesions of IgG4-RD has been emphasized so far. Recently, new players have been attracting attention in relation to the pathophysiology of this disease. For example, it has been pointed out that the counts of follicular helper T cells and peripheral helper T cells in peripheral blood each correlate with the numbers of affected organs and serum IgG4 levels. Although these findings suggest that autoimmune abnormality would cause the development of IgG4-related disease, T cell independent immune reaction has also been suggested to be involved in the pathogenesis of IgG4-RD. Accordingly, the question of whether IgG4-RD is an autoimmune disease is an issue that must be elucidated when considering the disease management

Prof. Dr. Kazunori Yamada
Prof. Dr. Hiroki Takahashi
Prof. Kenji Notohara
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Immuno is an international peer-reviewed open access quarterly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 1200 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.