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Intravenous IgG Reduces Pathogenic Autoantibodies, Serum IL-6 Levels, and Disease Severity in Experimental Bullous Pemphigoid Models

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Title: Intravenous IgG Reduces Pathogenic Autoantibodies, Serum IL-6 Levels, and Disease Severity in Experimental Bullous Pemphigoid Models
Other Titles: IVIG reduces disease severity in experimental BP
Authors: Sasaoka, Tetsumasa Browse this author
Ujiie, Hideyuki Browse this author →KAKEN DB
Nishie, Wataru Browse this author →KAKEN DB
Iwata, Hiroaki Browse this author →KAKEN DB
Ishikawa, Makoto Browse this author
Higashino, Hiroshi Browse this author
Natsuga, Ken Browse this author →KAKEN DB
Shinkuma, Satoru Browse this author →KAKEN DB
Shimizu, Hiroshi Browse this author →KAKEN DB
Issue Date: Jun-2018
Publisher: Elsevier
Journal Title: Journal of investigative dermatology
Volume: 138
Issue: 6
Start Page: 1260
End Page: 1267
Publisher DOI: 10.1016/j.jid.2018.01.005
PMID: 29391250
Abstract: Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by autoantibodies to COL17. Currently, systemic corticosteroids are used as first-line treatments for BP; alternatively, intravenous administration of high-dose IgG (IVIG) has been shown to be effective for patients with steroid-resistant BP in clinical practice. However, the effect of IVIG on BP has not fully been investigated. To examine the effects and mechanisms of action of IVIG against BP, we performed IVIG experiments using two experimental BP mouse models. One is a passive-transfer BP model that reproduces subepidermal separation in neonatal mice by the passive transfer of IgGs against COL17, such as polyclonal or monoclonal mouse IgG or IgG from BP patients. The other is an active BP model that continuously develops a disease phenotype in adult mice. IVIG decreased pathogenic IgG and the disease scores in both models. Injected IVIG distributed throughout the dermis and the intercellular space of the lower epidermis. Notably, IVIG inhibited the increase of IL-6 in both models, possibly by suppressing the production of IL-6 by keratinocytes. These results suggest that the inhibitory effects of IVIG on BP are associated with the reduction of pathogenic IgG and the modulation of cytokine production.
Rights: © 2018. This manuscript version is made available under the CC-BY-NC-ND 4.0 license
Type: article (author version)
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 氏家 英之

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