Making History: First Drug Approved for Scleroderma-Associated Interstitial Lung Disease

According to a story from, the drug nintedanib (marketed as Ofev) has officially been approved by the US Food and Drug Administration (FDA) as a treatment for interstitial lung disease associated with scleroderma. This makes the drug the first officially approved treatment for this rare form of lung disease. The announcement is great news for patients with scleroderma, as lung complications are a major source of problems for these patients.

About Scleroderma

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 Sclerderma-Associated Interstitial Lung Disease

Interstitial lung disease is characterized by scarring and thickening of affected tissues, which are usually the membranes or spaces around the lung’s air sacs. This process begins to impede the lungs’ functional capability. It will come as no surprise that lung disease is a major factor for patients with scleroderma that can cause a patient’s condition to worsen drastically. In fact, interstitial lung disease is the most common cause of death for these patients. 

A Better Chance

A placebo controlled clinical trial that included a total of 576 scleroderma patients with interstitial lung disease revealed that Ofev was able to considerably slow the progression of lung function decline. Ofev was originally approved for idiopathic pulmonary fibrosis in 2014. This is another lung disease that shares similar characteristics to the lung disease seen in scleroderma patients.

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