BAFF-driven B cell hyperplasia underlies lung disease in common variable immunodeficiency.

ABSTRACT:

Background

Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency and is frequently complicated by interstitial lung disease (ILD) for which etiology is unknown and therapy inadequate.

Methods

Medical record review implicated B cell dysregulation in CVID ILD progression. This was further studied in blood and lung samples using culture, cytometry, ELISA, and histology. Eleven CVID ILD patients were treated with rituximab and followed for 18 months.

Results

Serum IgM increased in conjunction with ILD progression, a finding that reflected the extent of IgM production within B cell follicles in lung parenchyma. Targeting these pulmonary B cell follicles with rituximab ameliorated CVID ILD, but disease recurred in association with I

SUBMITTER: Maglione PJ 

PROVIDER: S-EPMC6483510 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature