Discovering significant breakthroughs in fibrosis.

FIBROSIS-RELATED DISEASES

Diseases characterized by uncontrolled, progressive fibrosis include idiopathic pulmonary fibrosis (IPF), non-alcoholic steatohepatitis (NASH) and systemic sclerosis (SSc).

Idiopathic Pulmonary Fibrosis (IPF)

IPF is a rare, highly debilitating and poorly understood disease. In the US it’s estimated that there are ~200k IPF patients and is a similar number in the EU. Most commonly affecting individuals over 50 years old, IPF is characterized by interstitial pneumonia and chronic progressive lung fibrosis causing a continuous decline in lung function. Disease progression is unpredictable and is often rapid. Unfortunately, the disease’s early genesis is unknown, and there are no tests to identify patients that are at greatest risk of developing IPF.

Non-Alcoholic Steatohepatitis (NASH)

NASH affects 350 million people worldwide, 15 million in the U.S. alone. It is a growing public health threat in which about one-third of patients have liver fibrosis and it is estimated that 25% of them will develop cirrhosis. Based on current projections, NASH is predicted to become the leading cause of liver transplantation by 2020. Unfortunately, there are no therapies available to prevent or treat liver fibrosis.

Systemic Sclerosis (SSc) – Interstitial Lung Disease (ILD)

SSc-ILD is a rare and chronic condition characterized by organ fibrosis (including hardening of the skin) and vasculopathy. ILD and pulmonary arterial hypertension are severe manifestations of SSc and when present, increase mortality significantly. ILD in SSc is often associated with a decline in lung function within the first several years of lung disease onset.

References

General Information about Pulmonary Fibrosis, 2015, American Thoracic Society.

GlobalData

Idiopathic Pulmonary Fibrosis, Published August 17, 2020. NIH – US National Library of Medicine, Genetics Home Reference.

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