BAFF inhibition attenuates fibrosis in scleroderma by modulating the regulatory and effector B cell balance

Journal: Science Advances

Published: 2018-07-01

DOI: 10.1126/sciadv.aas9944

Affiliations: 5

Authors: 12

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Research Highlight

Targeted immune therapy for scleroderma



Targeting only the B-cells in the immune system that contribute to scleroderma, and not those B-cells that serve a protective function, could help people with this rare connective tissue disorder, for which there isn’t an approved therapy.

Using a mouse model of scleroderma, a team led by scientists at Kanazawa University found that disease effects such as skin hardening and lung scarring were less prominent among animals lacking the ‘effector’ B-cells needed to produce immune-stimulating molecules. In contrast, these symptoms were exacerbated in mice without ‘regulatory’ B-cells, which quash immune responses.

The researchers found that a protein called BAFF normally drives the disease by activating effector B-cells and suppressing regulatory B-cells. A BAFF-inhibiting drug had the opposite effect in the mice: it restored a healthy B-cell balance and mitigated symptoms.

These findings suggest that a BAFF-targeted antibody therapy that is already approved for treating lupus should be studied for the treatment of scleroderma patients.

Supported content

  1. Science Advances 4, eaas9944 (2018). doi: 10.1126/sciadv.aas9944
Institutions Share
Division of Molecular Pathology of Skin, KU, Japan 0.75
Research Institute for Biomedical Sciences (RIBS), TUS, Japan 0.08
Faculty / Graduate School of Medical Sciences, University of Fukui, Japan 0.08
Faculty of Medicine, University of Tsukuba, Japan 0.08
Core Research for Evolutional Science and Technology (CREST), JST, Japan 0