Disease Research Areas

Interstitial Lung Disease

Interstitial lung disease (ILD) affects lung function by thickening the lung interstitium due to excessive production and accumulation of collagen, resulting in a wide variety of symptoms.

The interstitium is a lacy network of body tissue that supports the alveoli, which are like small grape-like sacs, and contains capillaries that facilitate gas exchange between blood and air. The potential causes of interstitial lung disease are inflammation, scarring, and edema.

There are many types of interstitial lung disease, the most common of which are:

• Infections caused by bacteria, viruses, and infectious bacteria
• Idiopathic pulmonary fibrosis (IPF) with an unknown cause
• Nonspecific interstitial pneumonia (NSIP) associated with autoimmune conditions such as rheumatoid arthritis (RA) and scleroderma (SD), and unspecified interstitial fibrosis pneumonia is also classified as interstitial pneumonia. In some cases, interstitial lung disease associated with connective tissue disease (CTD-ILD) is also classified as this.

The disease and progression process are related to the TGF-beta (fibroblast transformation and promoting factor), VEGF (vascular endothelial growth factor), PDGF (platelet-derived growth factor), and FGF (fibroblast growth factor) signaling pathways, but the intracellular mechanism of collagen overproduction and accumulation remains unclear.

Before pirfenidone was approved for idiopathic pulmonary fibrosis in China, the United States, Europe, and Japan, steroids were the only treatment, but their effectiveness was questionable, and they were accompanied by serious side effects.

Currently, the Company is continuing development for the expansion of indications for pirfenidone (Etuary®] [Chinese: 艾思瑞]) beyond idiopathic pulmonary fibrosis, particularly for interstitial lung disease associated with connective tissue diseases in China.