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

Go to article

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
Kanazawa University (KU), Japan 0.75
Tokyo University of Science (TUS), Japan 0.08
University of Fukui, Japan 0.08
University of Tsukuba, Japan 0.08
Japan Science and Technology Agency (JST), Japan 0