According to a story from American Nurse Today, the US Food and Drug Administration (FDA) approved a treatment on September 6th, 2019 for interstitial lung disease associated with scleroderma, a rare autoimmune disease that affects connective tissue. Lung disease is a major problem for patients with scleroderma and ultimately is a major contributor to disease progression and lethality. The drug in question is called nintedanib (marketed as Ofev) which has been approved since 2014 as a treatment for idiopathic pulmonary fibrosis, another form of rare interstitial lung disease.
Scleroderma, which is also referred to as systemic sclerosis, describes a group of autoimmune diseases that can cause system-wide effects in the most severe cases. The mechanism of this disease is believed to be an autoimmune response in which the immune system mistakenly attacks body tissue. Some factors that may contribute to triggering the autoimmune response include mutations of the HLA genes and exposure to certain materials, such as certain solvents, white spirits, ketones, and silica. Symptoms are broad ranging and systemic, including kidney failure, erectile dysfunction, fatigue, stroke, headaches, facial pain, congestive heart failure, skin abnormalities, high blood pressure, chest pain, indigestion, and many more. Treatments are varied and depend on the symptoms, but most patients take medications in an attempt to suppress the autoimmune response. In severe cases, life expectancy is around 11 years from onset. To learn more about scleroderma, click here.
About Interstitial Lung Disease
Interstitial lung disease refers to lung disease that affects a certain membrane called the interstitium, which surrounds the lung’s air sacs. This tissue gradually thickens over time, eventually turning into scar tissue. The result is progressive loss of lung function; this eventually becomes fatal when the lungs are unable to provide sufficient oxygen to the heart. Research suggests that over half of scleroderma patients are affected by lung disease.
The approval of this drug should make a significant impact on survival and quality of life for patients affected by interstitial lung disease and marks a substantial step forward in the treatment of scleroderma as a whole.