Systemic Sclerosis Patients with ILD Being Asked to Participate in Phase 3 Study of OFEV

Boehringer Ingelheim recently announced the enrollment of the first patient in its Phase 3 clinical trial, called SENSCIS (Safety and Efficacy of Nintedanib in Systemic Sclerosis), evaluating the safety and efficacy of the company’s product nintedanib (OFEV) in patients with systemic sclerosis and interstitial lung disease.

Systemic sclerosis is a rare, chronic autoimmune disease in which the body’s own immune system attacks healthy tissues, resulting in a hardening and tightening (scarring, fibrosis) of the skin and connective tissues due to excessive collagen deposition. The disease usually affects the skin, but it can also affect organs such as the lungs, heart, blood vessels, kidneys, and the digestive tract. When the lungs are affected by this fibrotic process, the condition is called systemic sclerosis-interstitial lung disease (SSc-ILD). ILD presence in SSc patients is usually linked to a poorer prognosis and currently has no approved treatments.

Nintedanib is a tyrosine-kinase inhibitor (TKI) that targets growth factors, like the vascular endothelial growth factor receptor (VEGFR), thought to be involved in pulmonary fibrosis and pathogenesis. Nintedanib has been shown to be safe and effective in reducing disease progression and exacerbations in patients with idiopathic pulmonary fibrosis (IPF), and is an U.S. Food and Drug Administration (FDA)-approved treatment for the condition. Boehringer Ingelheim is now exploring the potential therapeutic effect of nintedanib in serious fibrotic lung conditions associated with rare disorders such as systemic sclerosis.

“Nintedanib, which is marketed as OFEV®, is approved for a rare lung disease called idiopathic pulmonary fibrosis, or IPF, and has been shown to slow disease progression as measured by annual rate of decline in lung function. Because SSc-ILD and IPF share similarities in how the underlying lung scarring, or fibrosis, forms in people with the disease, we are evaluating the impact of nintedanib on lung fibrosis associated with systemic sclerosis,” said Dr. Danny McBryan, vice president, Clinical Development and Medical Affairs, Respiratory, Boehringer Ingelheim Pharmaceuticals, Inc., in a press release.

“People affected by systemic sclerosis with interstitial lung disease are often young, between 25 and 55 years old, and are faced with considerable disability related to the systemic nature of scleroderma. They often have significant shortness of breath and cough from lung disease and severe pain and contractures as a consequence of their skin involvement. This occurs during years when they are deeply engaged in building careers and caring for family,” said Dr. Kristin Highland from Cleveland Clinic Foundation. “There are no approved treatments, and few drugs have been assessed in clinical trials for this disease. The SENSCIS™ trial will help to further inform the medical community about this disease and whether nintedanib could be an effective therapy.”

The SENSCIS trial (NCT02597933) is a randomized, double-blind, placebo-controlled study to evaluate the safety and efficacy of nintedanib (150 mg twice daily, for at least 52 weeks and up to 100 weeks) in patients with SSc-ILD. The trial’s primary endpoint is disease progression, assessed by the annual rate of decline in lung forced vital capacity (FVC), and secondary endpoints include changes in skin thickness and health-related quality of life.

Dr. Highland explained in more detail to Pulmonary Fibrosis News the clinical endpoints of this trial. “The SENSCIS trial will primarily examine respiratory function, as this is globally accepted for assessment of treatment effects in patients with lung fibrosis, and the chosen endpoint (forced vital capacity decline) is easy to obtain and part of the usual examination of patients with SSc-ILD,” she said. “The study also will examine the impact of nintedanib on skin manifestations through the modified Rodnan Skin Score as a secondary endpoint. Other endpoints in the study include effects on digital ulcers, vascular effects will be evaluated in an exploratory manner.”

“The community has been waiting for treatment options for people with systemic sclerosis who have developed interstitial lung disease,” said Robert Riggs, CEO of Scleroderma Foundation. “The initiation of Phase III research such as the SENSCIS trial is an important step forward for people living with the disease and their families.”

The trial is currently recruiting participants in the United States and several European countries. Researchers expect to enroll 520 patients. For more information about the trial and how to participate, please visit this link.

“The SENSCIS™ study is another milestone in our ongoing commitment to furthering science that addresses the unmet needs of people affected by rare diseases and serious respiratory conditions, including fibrotic lung diseases,” concluded Dr. McBryan. When asked by Pulmonary Fibrosis News whether Boehringer Ingelheim is planning to assess nintedanib’s therapeutic potential in other medical conditions, Dr. McBryan noted, “We are currently investigating the potential use of nintedanib in conditions that commonly occur with fibrotic lung conditions, such as systemic sclerosis and other progressive fibrosing interstitial lung diseases.”

The completion of clinical trials can sometimes be hindered by the lack of participants, especially in studies of rare diseases. Knowing this, Dr. Highland emphasized to Pulmonary Fibrosis News, “Virtually every treatment physicians use today is the result of clinical research, and patient participation in clinical studies is extremely valuable and an important contribution to the understanding of how to treat a disease. It also gives patients an opportunity to access new therapies over what is available outside the clinical study. In the case of systemic sclerosis with interstitial lung disease, since there are no approved treatments, the SENSCIS study allows exploration of a treatment that may prove to be effective.”