Ofev (nintedanib), manufactured by Boehringer Ingelheim, is being investigated for its ability to treat interstitial lung disease (ILD), a group of disorders in which tissues and spaces around the lungs’ air sacs (the interstitium) become inflamed and damaged. ILD is known to develop in people with systemic scleroderma.

Ofev is approved by the U.S. Food and Drug Administration to treat idiopathic pulmonary fibrosis (IPF). The FDA has also  granted it fast track and orphan drug designation as a possible treatment of systemic scleroderma with associated ILD.

How Ofev works

Ofev belongs to a class of medications known as tyrosine kinase inhibitors. It works by targeting proteins called growth factor receptors, known to be involved in pulmonary fibrosis, a type of ILD. The specific receptors it targets and blocks are called the platelet-derived growth factor receptor (PDGFR), fibroblast growth factor receptor (FGFR), and vascular endothelial growth factor receptor (VEGFR), as these are found in excessive amounts in IPF patients.

By blocking the signaling pathways  involved in fibrotic processes, Ofev works to slow IPF progression and the decline in a patient’s lung function.

Ofev in trials for scleroderma-related ILD

The safety and efficacy of the Ofev — under its chemical name, nintedanib — in treating scleroderma-related pulmonary fibrosis is currently being tested in a Phase 3 clinical trial (NCT02597933) called SENSCIS.

In this global, randomized and double-blind trial, more than 520 scleroderma patients with associated fibrotic lung disease are being given either nintedanib capsules at a dose of 150 mg twice a day or placebo for at least 52 weeks. Researchers are analyzing the medicine’s effectiveness in slowing disease progression by assessing the rate of lung function decline after one year of treatment.  Specifically, they are measuring a patient’s forced vital capacity (FVC), the amount of air that can be forcibly exhaled from the lungs after taking the deepest possible breath. Other, secondary trial goals include changes in tissue thickness and health-related quality of life.

SENSCIS is due to conclude in November 2018.

About 400 patients in the Phase 3 trial are being invited to enroll in its long-term extension study (NCT03313180), which will evaluate nintedanib’s safety by looking at recorded adverse events during up to 34 months of treatment. This study is open label, meaning all patients will be given oral nintedanib twice daily, and is expected to end in July 2021.

Other details

Common side effects of Ofev use are diarrhea, nausea, abdominal pain, vomiting, liver enzyme elevation, decreased appetite, headache, weight loss, and hypertension.

Blood tests to assess liver function are frequently needed in people taking Ofev to verify that the medicine is not harming the liver.  Women of child-bearing age should not take Ofev, as it can cause birth defects and harm to a fetus.

 

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