Clonal Hematopoiesis Instructs Maladaptive Tissue Repair to Promote Fibrosis
Dongzhu Li, Ana C. Viñado, Paula García‐OlloquiiD, Sydney B. MontesiiD, Barry S. SheaiD, Leonard Christian, Iria Vázquez-Urio, Paula Aguirre-RuiziD, Beñat AricetaiD, Manuela Neubert, Ashutosh Tripathy, Niloy Barua, Pablo Valera, Laura Vera, Eva Petri, Antje PrasseiD, Benjamin SeeligeriD, Amaia Vilas, Patxi San Martin, María José CalasanziD, Felipe PrósperiD, Peter Grant MilleriD, Werner SeegeriD, Florian Heinrich HeideliD, Jonas C. SchuppiD, , Ana Pardo–SagantaiD
Abstract
Tissue repair is increasingly recognized as a systemic process influenced by age-associated changes beyond the injured organ itself. Clonal hematopoiesis of indeterminate potential (CHIP), a common consequence of somatic evolution in hematopoietic stem cells, has been linked to inflammatory disorders, yet whether it directly regulates tissue remodeling remains unclear. Here, we integrate population genomics, preclinical models, and human lung analyses to examine the role of CHIP in fibrotic lung disease. In large cohorts, idiopathic pulmonary fibrosis (IPF) was associated with a distinct CHIP mutational spectrum enriched for non-DNMT3A variants and for larger mutant clones. In mouse models, hematopoietic mutations exacerbated bleomycin-induced fibrosis and reprogrammed macrophages toward inflammatory, profibrotic states, including expansion of a distinct, injury-responsive SPP1+ population conserved in human disease. CHIP-associated macrophages were sufficient to directly promote fibroblast activation and alter epithelial differentiation, linking hematopoietic genotype to parenchymal remodeling. Consistently, a CHIP-derived macrophage transcriptional signature predicted adverse outcomes in independent IPF cohorts. Notably, immune and epithelial alterations were detectable even in the absence of overt injury, indicating that CHIP establishes a primed tissue environment permissive for maladaptive repair. Together, these findings identify clonal hematopoiesis as a systemic regulator of tissue repair and demonstrate that somatic evolution in blood can actively instruct organ remodeling through immune-parenchymal interactions. This framework supports the possibility that disease-associated selective pressures may shape clonal architecture with functional consequences for organ health.
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