Fibrosis


 
Fibrosis of the airway and lung tissue is the abnormal scarring of normal tissue that leads to loss of tissue and organ function. At the present time, there are no treatments for fibrotic disorders except transplant. Fibrotic scarring can occur in the airways or in the lung tissue. The severity of the fibrosis can range from mild, which is often asymptomatic, to severe, which is life limiting. 
 

 

There are over 140 known causes of pulmonary fibrosis. The common theme is that they create a chronic injury in the lung tissue that leads to the scarring reaction. Pulmonary fibrosis is a common complication of radiation or chemotherapy for cancers, and limits the amounts of radiation or drugs that can be administered. In some cases, the cause is unknown (idiopathic).

LRRI is developing animal models of fibrosis to study the etiology of the disorder and the evaluation of potential therapeutics that could either prevent the fibrosis or slow its progression. Patients diagnosed with pulmonary fibrotic disorders such as idiopathic pulmonary fibrosis often have a prognosis similar to that of lung cancer patients, with a mean survival time of 12-24 months post-initial diagnosis. However, other forms of pulmonary fibrosis, such as those induced by adverse chemotherapeutic drug reactions, radiation treatment or exposure to chemical weapon agents could be responsive to appropriate therapies as treatment could begin before the fibrotic mechanisms have had sufficient time to become refractory to drug treatment.

Contract research services include animal models of drug-, radiation-, and chemical-induced forms of lung fibrosis. Both in vitro and in vivo testing of anti-fibrotic therapeutics are available.

 

Current Research Interests

  • Radiation-induced pulmonary fibrosis
  • Bleomycin-induced pulmonary fibrosis, with an improved aerosol delivery model of bleomycin
  • Immune mediated fibrosis as a result of transplant
  • Regulatory roles of TGFβ and CTGF in fibrotic scarring

Key Capabilities

  • Radiation and other models in animals for studies of fibrotic mechanisms and therapeutic validation
  • In vitro models for therapeutic evaluation of anti-fibrotic therapeutics
  • Tracheal transplant model of allogenic versus syngenic grafts
  • Cell and molecular biological, proteomic and immunologic analysis of cytokines, matrix proteins, and other markers of fibrotic scarring
  • Sophisticated histopathology, morphometry, immunohistochemistry, and in situ hybridization for analysis of lung tissues

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