FDA Approves Ofev for Sarcoidosis and Other Interstitial Lung Diseases

Boehringer Ingelheim‘s Ofev (nintedanib) has been approved in the U.S. as the first treatment for chronic fibrosing interstitial lung diseases (ILDs) with a progressive phenotype.

This approval by the United States Food and Drug Administration (FDA) includes sarcoidosis, autoimmune ILDs, unclassifiable ILDs, chronic hypersensitivity pneumonitis, myositis, Sjogren’s syndrome, coal worker’s pneumoconiosis, and some forms of interstitial pneumonia. All these conditions commonly involve fibrosis (scarring) in the lungs.

“Today’s approval marks a major advancement in ILD research, and is an important milestone just six months after the approval of Ofev to slow the rate of decline in pulmonary function in patients with SSc-ILD,” Thomas Seck, MD, senior vice president of medicine and regulatory affairs at Boehringer Ingelheim, said in a press release.

Ofev is an inhibitor of tyrosine kinase, and works by inhibiting a number of molecular pathways involved in the formation of scarring.

It received breakthrough therapy designation from the FDA in 2019 for the treatment of ILDs. Ofev was first approved to treat idiopathic pulmonary fibrosis in 2014, and ILD associated with systemic sclerosis in 2019.

This new approval makes Ofev available for individuals with ILDs with worsening lung fibrosis over time (progressive phenotype).

“This approval provides a therapeutic option for many patients who did not have an approved treatment until today,” said Kevin Flaherty, MD, University of Michigan professor and lead investigator of the INBUILD clinical trial (NCT02999178).

The FDA decision was based on positive data from this Boehringer Ingelheim-funded Phase 3 trial, in which 663 participants with progressive ILDs were treated with Ofev (150 mg twice daily) or given a placebo.

Results showed that Ofev treatment significantly slowed the decline in lung function, as measured by forced vital capacity — the average annual rate of decrease was 80.8 ml per year with Ofev, compared to 187.8 ml per year with a placebo, which corresponds to a difference of 107 ml per year and a reduction in loss of lung function by 57%.

Ofev’s safety profile in the trial was consistent with that reported in previous studies. The treatment’s most common adverse side effects included diarrhea, followed by nausea, vomiting, abdominal pain, decreased appetite, weight loss, elevated levels of liver enzymes, headache, and hypertension (high blood pressure).

“Patients with a progressive form of chronic fibrosing ILDs may have symptoms that are similar to other respiratory illnesses, and that may delay getting an accurate diagnosis for patients,” said Greg Cosgrove, MD, chief medical officer of the Pulmonary Fibrosis Foundation. “The new indication for nintedanib provides a therapeutic option for physicians and their patients as there is now a treatment option that can help slow the decline in lung function.”

According to Boehringer, 18% to 32% of patients with ILDs develop chronic fibrosing disease with a progressive phenotype.

The company has a support program for patients prescribed Ofev called OPEN DOORS.