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INTERSTITIAL LUNG DISEASE
EmphyCorp has been granted Orphan Drug status by the FDA to develop N115 for the treatment of Interstitial Lung Disease. Interstitial lung diseases are typically characterized by a marked inflammation at the site of the lung injury.
This inflammatory process leads to further destruction of surrounding healthy lung tissue, and a continuation and expansion of the sites of inflammation.
This inflammatory process results in the production of reactive oxygen species, including superoxide anions and hydrogen peroxide, at the site of inflammation.
The functional changes reflect a restriction of airflow manifest by:
- Decreased vital capacity
- Decreased total lung capacity
- Decreased residual volume
- Decreased lung compliance
- Cyanosis (sign of severe hypoxemia; attributed to ventilation-perfusion mismatching)
- Late in the disease, one may see pulmonary hypertension due to destruction of the alveolar capillary bed
Nitric oxide is a known bronchodilator. It has been used successfully in this regard to treat patients with various pulmonary diseases, including the interstitial lung diseases.
Reactive oxygen species, especially superoxide anions, are known to compromise lung function by increasing bronchoconstriction’s.
As a result of the increasing inflammation and the production of reactive oxygen species, and the decrease in nitric oxide that occurs with the interstitial lung diseases, healthy tissue is damaged and lung function is compromised.
Sodium pyruvate is a reactive oxygen species (ROS) antagonist that has been shown to neutralize oxygen radicals (specifically lowering the overproduction of superoxide anions), reduce the production and level of other inflammatory agents including inflammatory cytokines like Interleukin 6, reduce hypoxemia and coughing and upregulate the synthesis of nitric oxide.
Sodium pyruvate also increases cellular levels of glutathione, a major cellular antioxidant, which is reduced dramatically in lung disease patients.