OFEV (nintedanib) Added To Updated International Treatment Guidelines for Idiopathic Pulmonary Fibrosis (IPF)

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by Charles Moore |

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Pulmonary Hypertension Functional Classification

Revised international treatment guidelines for Idiopathic Pulmonary Fibrosis (IPF) suggest use of OFEV (nintedanib), a proprietary drug from Ingelheim, Germany, based Boehringer Ingelheim Corporation for the treatment of the disease. This newly-added recommendation places high value on potential benefits of OFEV on patient-important outcomes such as slowing of disease progression measured by the rate of forced vital capacity (FVC) — the amount of air which can be forcibly exhaled from the lungs after taking the deepest breath possible — decline, and takes into account potential significant adverse effects and the expected cost of treatment.

The revised international evidence-based 2015 ATS/ERS/JRS/ALAT Clinical Practice Guideline: “Treatment of Idiopathic Pulmonary Fibrosis” — updates the 2011 guideline which suggests that clinicians use OFEV in patients with IPF. This recommendation is conditional, i.e.: meaning that clinicians must discuss preferences with their patients to help patients reach treatment decisions consistent with their values and preferences.

Members of the joint guidelines committee include representatives from an international coalition of leading respiratory societies including the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT).

“These guidelines, which update the 2011 iteration, focus on evaluation of clinical trial data in an effort to give healthcare providers an evidence-based set of recommendations for the treatment of IPF, says Imre Noth, M.D., a pulmonologist and Professor of Medicine and Director of the Interstitial Lung Disease Program at The University of Chicago. “Our priority is to provide quality care for patients living with this devastating disease. These new guidelines help to outline the role of OFEV in the treatment of IPF.”

Dr. Noth specializes in interstitial lung diseases (pulmonary fibrosis). Both in his practice seeing patients in clinics, as well as in the intensive care unit, he sees a wide variety of pulmonary patients ranging from emphysema to asthma and interstitial lung disease. Over the last half-decade, has concentrated his efforts on interstitial lung diseases with a special emphasis on idiopathic pulmonary fibrosis (IPF), allowing him to focus on the unique needs of this patient population.

Dr. Noth has participated as principal investigator in numerous clinical trials that focus predominantly on IPF, but also on severe asthma, and chronic obstructive pulmonary diseases (COPD), and is recognized in the field for his expertise. His recent research includes studying the relationship between genetics, genomics and outcomes in IPF, as well as in the conduct of novel clinical trials in IPF, and he is also a member of the National Institutes of Health sponsored IPF clinical research network, and hopes to continue conducting research leading to better understanding of the natural history of interstitial lung diseases and development of better therapies.

Idiopathic pulmonary fibrosis is a condition in which the lungs become progressively scarred over time. As a result, patients with IPF experience shortness of breath, cough, and have difficulty participating in everyday physical activities. Current, non-drug treatments for IPF include oxygen therapy, pulmonary rehabilitation, and lung transplant.

IPF is estimated to affect up to 132,000 people in the United States — most commonly men over the age of 65 — as compared for context to the number of people with diabetes, which affects over 29 million Americans, which demonstrates how uncommon IPF is. Indeed, most people have never heard of IPF, but recent studies show it is more common in the United States than has been traditionally thought, with about 50,000 new cases now diagnosed each year. Early diagnosis and proper care and treatment are critical to helping people with the condition.

One reason for the increase may be an aging population. “Older age is a well-known risk factor for IPF, a disease in which the lung tissue becomes progressively fibrotic, or scarred, leading to breathing difficulties and death within three to five years if a lung transplant isn’t possible,” says Ana L. Mora, M.D. , assistant professor in the Division of Pulmonary, Allergy and Critical Care Medicine and a member of the Heart, Lung, Blood and Vascular Medicine Institute (VMI) at The University of Pittsburgh in a release. “The cause of the disease is unknown, or idiopathic.”

“Until last year, treatment options for patients with IPF were limited. These updated international guidelines mark an important milestone in the care of IPF patients, and were very pleased that OFEV is included as a recommended treatment option for this devastating disease,” says Danny McBryan, M.D., vice president, Clinical Development and Medical Affairs, Respiratory, at Ridgefield, Connecticut-based Boehringer Ingelheim Pharmaceuticals, Inc.

As Dr. Bryant notes, There is currently no cure for IPF, however, OFEV (nintedanib) — a kinase inhibitor that blocks multiple pathways that may be involved in the scarring of lung tissue, may help slow the disease’s progression. In 3 out of 3 clinical trials of 1,231 patients with IPF, patients who took OFEV found it slowed the decline in lung function by approximately 50 percent compared with patients taking a placebo measured by FVC decline.

As more people learn about IPF, more support groups and information that can help are becoming available. OPEN DOORS is a resource that specializes in providing personal support and advice for people who have been prescribed OFEV capsules, where you can speak with a trained IPF nurse, receive information and brochures about IPF, and contact Clinical Educators trained to teach patients and caregivers one-on-one.

The international treatment guidelines committee continuously analyzes evidence as it becomes available and updates treatment recommendations accordingly, and this latest tranche of updated guidelines include OFEV data from two replicate Phase 3 trials (INPULSIS-1 and INPULSIS-2) involving more than 1,000 patients in 24 countries, and a Phase II trial (TOMORROW) involving 432 patients.

The U.S. Food and Drug Administration (FDA) approved OFEV for the treatment of idiopathic pulmonary fibrosis (IPF) on October 15, 2014, after granting the drug fast track, priority review, orphan product, and breakthrough designations. OFEV is one of the first FDA-approved drug treatments for IPF and the only kinase inhibitor approved to treat this disease.

The approval was based on findings from the Phase II TOMORROW trial (NCT00514683) and the Phase III INPULSIS trials (INPULSIS-1 and INPULSIS-2; NCT01335464 and NCT01335477) which were all randomized, double-blind, placebo-controlled trials comparing OFEV 150 mg twice daily to placebo for 52 weeks. Both INPULSIS trials were identically designed while the TOMORROW study design was similar.

OFEV’s manufacturer Boehringer Ingelheim Pharmaceuticals, Inc. , based in Ridgefield, Connecticut, is the largest U.S. subsidiary of Boehringer Ingelheim Corporation — one of the worlds’ 20 leading pharmaceutical companies — in 2014 achieving net sales of about $16.96 billion dollars (13.3 billion euros). R&D expenditure corresponds to 19.9 percent of Boehringer Ingelheim’s net sales. Headquartered in Ingelheim, Germany, the company operates globally with 146 affiliates and more than 47,000 employees. Since its founding in 1885, the family-owned company has been committed to researching, developing, manufacturing and marketing novel treatments for human and veterinary medicine, improving lives and providing services and support to patients and families.

Boehringer Ingelheim Pharmaceuticals, Inc.
U.S. Food and Drug Administration (FDA)
University of Chicago
The University of Pittsburgh

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Boehringer Ingelheim Pharmaceuticals, Inc.
University of Chicago
The University of Pittsburgh