Ofev Used to Successfully Treat Acute Episode of IPF, Prompting Call for Studies

An 84-year-old man diagnosed with an acute exacerbation of idiopathic pulmonary fibrosis (IPF) was successfully treated with Ofev (nintedanib) without antibiotics or steroids, according to a case study detailed in the journal Respirology Case Reports.

Ofev, an approved treatment in the United States and EU, is a tyrosine kinase inhibitor that works to prevent a rapid worsening of IPF symptoms.

The case study was in the report, “Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis,” published this month.

Acute exacerbations occur in some IPF patients for unknown reasons. There is no treatment for acute exacerbations, and the outcome is usually poor.

The patient was diagnosed with IPF in June 2014, and he was admitted to a hospital in February 2016 with dry cough and hypoxemia (a low level of oxygen in the blood). He was diagnosed with an acute exacerbation of IPF once other possible causes were excluded.

His predicted forced vital capacity (FVC) was 92.8% at a regular visit in December 2015, and it had dropped to 77.8% when he was hospitalized. FVC measures how much air the patient can expel after taking a deep breath, and is used to detect and evaluate lung disease.

There was no evidence of lung infection or congestive heart failure, and the man was treated with 300 mg of Ofev per day without antibiotics or corticosteroids. He was released from the hospital 11 days later and continued to take Ofev without significant side effects. Within two months, his FVC reached 89.8%.

High-resolution computed tomography (HRCT) — a special scanning technique used to diagnose many health problems — performed in January 2016 showed new areas of fibrous tissue in the man’s lungs. (Fibrous tissue in the lungs and the scarring it causes are responsible for the loss in oxygen absorption seen in IPF.) Chest HRCT done in March — after Ofev therapy — showed improvement in his fibrotic tissue.

Ofev blocks naturally occurring factors, called tyrosine kinases, by inhibiting their receptors. The drug targets fibroblast growth factor (FGF), platelet-derived growth factor (PDGF), and vascular endothelial growth factor (VEGF). All play a role in producing fibrous tissue in IPF.

“After exclusion of alternative causes, acute exacerbation of IPF was diagnosed and we started treatment with nintedanib of 300 mg/day. This resulted in the gradual improvement of his condition and HRCT findings without administering antibiotics or corticosteroids,” the team wrote.

“In summary, this case report suggests that nintedanib therapy may have possible benefits in acute exacerbation of IPF. Further experience and clinical studies are needed to verify this promising approach,” they wrote.