Title: Nintedanib for treating idiopathic pulmonary fibrosis when forced vital capacity is above 80% predicted (part-review of technology appraisal guidance 379) [ID4062]
Funder: Department of Health and Social Care

Abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease in which scarring (fibrosis) occurs. The cause of IPF is unknown although it is thought to be related to an abnormal immune response to an unknown cause. It is a difficult disease to diagnose and requires a multidisciplinary team. Most people with IPF experience symptoms of breathlessness, which may initially be only on exertion. Cough, with or without sputum, is a common symptom. Over time, these symptoms are associated with a decline in lung function, reduced quality of life, disability and death._x000D_ _x000D_ The median survival for people with IPF in the UK is approximately 3-4 years from the time of diagnosis.[1],[2] However, about 20-30% of people with IPF survive for more than 5 years.[1],[2] The rate of disease progression can vary greatly. Prognosis is difficult to estimate at the time of diagnosis and may only become apparent after a period of careful follow-up._x000D_ _x000D_ The prevalence rate of IPF is about 50 per 100,000 [3] with an incidence of approximately 9 per 100,000 per year [4] , which equates to around 6000 new diagnoses each year in the UK.[3],[4] The incidence is higher in men than women, and increases with age (85% of diagnoses are made in people aged over 70 years).[3] IPF co-exists with chronic obstructive pulmonary disease in around 8-15% of people._x000D_ _x000D_ The aim of treatment is to manage the symptoms and slow progression. NICE clinical guideline 163 on the diagnosis and management of suspected idiopathic pulmonary fibrosis recommends that best supportive care (including symptom relief, management of co-morbidities, withdrawal of therapies suspected to be ineffective or causing harm and end of life care) should be offered to people from diagnosis and be tailored according to disease severity, rate of progression and the person’s preference. If pharmacological treatment is considered appropriate, the guideline recommends use of pirfenidone and nintedanib if a person’s forced vital capacity (FVC) is between 50% and 80% of their expected value in line with recommendations in NICE technology appraisal guidance 504 and 379. Treatment with pirfenidone and nintedanib should be discontinued if there is evidence of disease progression (a decline in per cent predicted FVC of 10% or more within any 12 month period). Lung transplantation is an option if there are no contraindications._x000D_ _x000D_ The existing recommendations in NICE technology appraisal guidance 379 are optimised for a narrower population than covered by the marketing authorisation. Stakeholders have indicated that there may be new information to warrant an update of the current guidance which only recommends nintedanib when a person has a forced vital capacity (FVC) between 50% and 80% predicted._x000D_ _x000D_ References_x000D_ 1. NHS. Idiopathic pulmonary fibrosis. [online accessed March 2022]_x000D_ 2. Khor, Y et.al. (2020) Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review. European Respiratory Review. 29: 190158_x000D_ 3. British Lung Foundation (2016) The battle for breath – the impact of lung disease in the UK. [online accessed March 2022]_x000D_ 4. Hutchinson, J (2015) Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. European Respiratory Society. 46: 795-806

Lay abstract: To appraise the clinical and cost effectiveness of nintedanib within its marketing authorisation for treating idiopathic pulmonary fibrosis in people with a forced vital capacity above 80% predicted.

Research activity codes: 6.1 PHARMACEUTICALS
Health categories: RESPIRATORY