Fibrotic 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. It is estimated that there are fewer than 200,000 people with IPF in the United States, with 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, but often rapid, with a five-year survival rate of 50% and a median survival time of 3-5 years following diagnosis. Unfortunately, the disease’s early genesis is unknown, and there are no tests to identify people who are at greatest risk of developing IPF.

Non-Alcoholic Steatohepatitis (NASH)

NASH affects 350 million people worldwide and more than 16 million in the U.S. alone. It is a growing public health threat in which approximately one-third of patients have liver fibrosis, and it is estimated that 25% of them will develop cirrhosis (extensive liver fibrosis). NASH is predicted to become a leading cause of liver transplantation. 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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Stanton, 2016, Am J Kidney Dis. 63(2)(suppl 2):S3-S21.