Rare lung disease first drug! A new indication approved by Canada for the treatment of SSC ILD

November 29, 2019 / bioun / -- Boehringer Ingelheim, a German pharmaceutical giant, recently announced that Health Canada has approved the oral administration of small molecule tyrosine kinase inhibitor ofev (nintedanib, nedanib) to slow down the decline of pulmonary function in patients with systemic sclerosis related interstitial lung disease (SSC ILD) In the United States, ofev was approved by FDA in September this year to slow down the rate of pulmonary function decline in SSc ILD patients It is worth mentioning that ofev is the first and only drug approved in the United States and Canada to treat this rare lung disease Previously, ofev has been approved to treat patients with idiopathic pulmonary fibrosis (IPF) in more than 70 countries It has been proved that the drug can slow down the progress of IPF by reducing the annual decline rate of lung function (as measured by forced vital capacity [FVC]) IPF and SSC ILD are pulmonary fibrosis Interstitial lung disease (ILD) is the leading cause of systemic sclerosis (SSC), accounting for 35% of all disease-related deaths This approval will change the treatment mode of SSC ILD and provide an opportunity for patients to slow down the progress of pulmonary function decline "This is the first drug approved in Canada to treat sscild, which can slow down the decline of lung function and bring hope to patients and their relatives facing this devastating disease," said Uli Br? DL, vice president of medical and regulatory affairs of Brigham Ingelheim Canada This approval was supported by positive evidence from the phase III senscis study, which showed that ofev significantly slowed the decline of pulmonary function in this patient population and demonstrated the company's commitment to this rare disease population " The approval is based on data from the phase III clinical study senscis ((nct02597933), the largest SSC ILD study ever conducted The study is a double-blind, randomized, placebo-controlled study involving 576 patients from 194 sites in 32 countries In the study, patients were randomly assigned to receive a second daily oral dose of ofev (150mg) or placebo The primary end point was the annual rate of FVC decline in SSc ILD patients The results showed that after one year (52 weeks) of treatment, compared with placebo, ofev significantly reduced the pulmonary function of SSC ILD patients by 44% (FVC adjusted annual decline rate: - 52.4 ml / year vs - 93.3 ml / year, absolute difference: 41.0 ml / year [95% CI: 2.9-79.0], P = 0.04) In this study, the overall safety profile observed in the ofev treatment group was consistent with the known safety profile of the drug Pneumonia was the most common serious adverse event in patients treated with ofev (2.8% in the ofev group vs 0.3% in the placebo group) 34% of patients treated with ofev had adverse reactions leading to permanent dose reduction, compared with 4% in the placebo group Diarrhea is the most common side effect of permanent dose reduction of ofev Prescription information of ofev includes warning information for patients with moderate or severe liver injury, patients with elevated liver enzymes and drug-induced liver injury, and patients with gastrointestinal diseases Ofev may also cause fetal fetal toxicity leading to fetal injury, arterial thromboembolism events, bleeding, and gastrointestinal perforation P-gp and CYP3A4 inhibitors may increase the exposure of ofev Patients taking these inhibitors should closely monitor the tolerance of ofev Common side effects of ofev include diarrhea, nausea, abdominal pain, vomiting, elevated liver enzymes, decreased appetite, headache, weight loss and high blood pressure (hypertension) Systemic sclerosis (SSC), commonly known as scleroderma, is a rare autoimmune disease characterized by thickening and scarring of connective tissue It is estimated that the disease affects 100000 people in the United States, 40000 people in Canada and 2.5 million people worldwide Fibrosis is a hallmark of the disease, affecting the skin and internal organs, including the lungs Interstitial lung disease (ILD) is one of the most common diseases, which can make people weak and even life-threatening About 25% of the patients had obvious lung involvement within 3 years after diagnosis, and ILD was the main cause of death in SSc patients SSC ILD is a kind of progressive lung disease, and the lung function decreases with time It is estimated that about half of SSc patients have SSC ILD Source of the original text: Health Canada app roads ofev (nintedanib), the first and only therapy in Canada to slow the rate of decline in pulmonary function in patients with systematic sciences associated ILD