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Background <p>Liver cirrhosis and pulmonary fibrosis are fibrotic disorders that arise from chronic wound-healing processes in response to persistent injury. Despite involving different organs, both conditions share pathophysiologic mechanisms, such as fibroblast-to-myofibroblast transformation, extracellular matrix deposition, and impaired remodeling. These overlapping pathways suggest a common fibrogenic network that extends beyond the organs.</p> Main Text <p>In cirrhosis, hepatocyte necrosis and chronic inflammation activate hepatic stellate cells that later differentiate into myofibroblasts and produce an altered extracellular matrix. Initially, matrix metalloproteinases counterbalance fibrosis. However, their activity is progressively inhibited by tissue inhibitors of metalloproteinases leading to excessive scarring, and regenerative nodules. Similarly, persistent alveolar epithelial injury disrupts regenerative capacity, causing maladaptive repair in pulmonary fibrosis. Fibrogenic mediators, particularly transforming growth factor-β and platelet-derived growth factor, drive fibroblast-to-myofibroblast differentiation and epithelial-mesenchymal transition. The resulting dense extracellular matrix creates a positive feedback loop that perpetuates fibrosis, leading to microscopic honeycombing and the histopathological pattern of usual interstitial pneumonia. Genetic and environmental factors cause the development of both conditions. Telomerase mutations and shortened telomeres predispose to cirrhosis and idiopathic pulmonary fibrosis. Smoking, hepatitis B and C are environmental factors for cirrhosis. On the other hand, epstein-barr virus, cytomegalovirus, and hepatitis C virus are responsible for pulmonary fibrosis. Both diseases promote vasoconstriction, leading to portal hypertension in the liver and pulmonary hypertension in the lungs. Transforming growth factor-β plays a dual role in carcinogenesis, acting as a tumor suppressor in early stages but later acquires tumor-promoting properties in both diseases.</p> Conclusion <p>Cirrhosis and pulmonary fibrosis share molecular and cellular processes leading to fibrosis, vascular remodeling and malignant transformation in both diseases. Recognizing these overlapping mechanisms may help us better understand disease processes and guide development of targeted treatments.</p>

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The intersection of liver cirrhosis and pulmonary fibrosis

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年12月26日

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  • Esref Alperen Bayraktar,
  • Mary Salvatore

摘要

Background

Liver cirrhosis and pulmonary fibrosis are fibrotic disorders that arise from chronic wound-healing processes in response to persistent injury. Despite involving different organs, both conditions share pathophysiologic mechanisms, such as fibroblast-to-myofibroblast transformation, extracellular matrix deposition, and impaired remodeling. These overlapping pathways suggest a common fibrogenic network that extends beyond the organs.

Main Text

In cirrhosis, hepatocyte necrosis and chronic inflammation activate hepatic stellate cells that later differentiate into myofibroblasts and produce an altered extracellular matrix. Initially, matrix metalloproteinases counterbalance fibrosis. However, their activity is progressively inhibited by tissue inhibitors of metalloproteinases leading to excessive scarring, and regenerative nodules. Similarly, persistent alveolar epithelial injury disrupts regenerative capacity, causing maladaptive repair in pulmonary fibrosis. Fibrogenic mediators, particularly transforming growth factor-β and platelet-derived growth factor, drive fibroblast-to-myofibroblast differentiation and epithelial-mesenchymal transition. The resulting dense extracellular matrix creates a positive feedback loop that perpetuates fibrosis, leading to microscopic honeycombing and the histopathological pattern of usual interstitial pneumonia. Genetic and environmental factors cause the development of both conditions. Telomerase mutations and shortened telomeres predispose to cirrhosis and idiopathic pulmonary fibrosis. Smoking, hepatitis B and C are environmental factors for cirrhosis. On the other hand, epstein-barr virus, cytomegalovirus, and hepatitis C virus are responsible for pulmonary fibrosis. Both diseases promote vasoconstriction, leading to portal hypertension in the liver and pulmonary hypertension in the lungs. Transforming growth factor-β plays a dual role in carcinogenesis, acting as a tumor suppressor in early stages but later acquires tumor-promoting properties in both diseases.

Conclusion

Cirrhosis and pulmonary fibrosis share molecular and cellular processes leading to fibrosis, vascular remodeling and malignant transformation in both diseases. Recognizing these overlapping mechanisms may help us better understand disease processes and guide development of targeted treatments.