Abstract
Autoimmunity Systemic sclerosis (SSc) is an autoimmune disease associated with fibrosis and serious complications including pulmonary arterial hypertension (PAH). Abnormal B cell responses have been associated with SSc pathogenesis. De Bourcy et al. analyzed immunoglobulin heavy chain transcripts of SSc-PAH patients enrolled in a clinical study of B cell depletion. SSc-PAH was associated with several B cell development anomalies, particularly underuse of the IGHV2-5 segment and B cell homeostasis abnormalities. Depletion temporarily reversed these anomalous SSc-PAH disease signatures, and the rate of naive B cell replenishment could be estimated from baseline measurements. These results define antibody signatures associated with SSc-PAH and reveal how B cell depletion shapes the antibody repertoire during reconstitution. Sci. Immunol. 2 , eaan8289 (2017).
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