Idiopathic pulmonary fibrosis in the UK: analysis of the British Thoracic Society electronic registry between 2013 and 2019

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and terminal interstitial lung disease (ILD) with a median survival of 3-5 years. The British Thoracic Society (BTS) established the UK IPF Registry in 2013 as a platform to collect data on clinical characteristics, treatments and outcomes for this cohort in the UK. Between 1 January 2013 and 31 October 2019, 2474 cases were registered. Most patients were male (79%) with a mean±sd age of 74±8.3 years and 66% were ex-smokers. Over time we observed an increase in the number of patients aged over 70 years. However, we have not seen a trend towards earlier presentation as symptoms of breathless and/or cough were present for >12 months in 63% of the cohort. At presentation, mean±sd % predicted forced vital capacity (FVC) was 78.2±18.3%, median 76.2% (interquartile range (IQR) 22.4%) and transfer factor of the lung for carbon monoxide (T _LCO) 48.4±16.0, median 47.5 (IQR 20.1). Most cases were discussed at an ILD multidisciplinary meeting, with an increase over this time in the number of cases reported as having possible usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) thorax. We noted a reduction in the number of patients undergoing surgical lung biopsy or bronchoalveolar lavage. Although more patients were prescribed anti-fibrotic therapies from 2013 to 2019, 43% were ineligible for treatment based upon National Institute for Health and Care Excellence (NICE) prescribing criteria. Hypertension, ischaemic heart disease, diabetes mellitus and gastro-oesophageal reflux were the most common comorbidities. In conclusion, we have presented baseline demographics as well as diagnostic and treatment strategies from the largest single-country IPF registry, reflecting changes in UK practices over this period.

FIGURE 1

Comorbidities in patients with idiopathic pulmonary fibrosis. Data presented are the reported patient comorbidities (% of patients) up to October 31, 2019. IHD: ischaemic heart disease; GORD: gastro-oesophageal reflux disease; PE/DVT: pulmonary embolism/deep vein thrombosis. ^#: Over 200 separate conditions were listed under “other comorbidities” including osteoarthritis, hypothyroidism, several cardiac disorders (the most common included aortic stenosis, atrial fibrillation and cardiomyopathy), and a number of cancers (the most common included prostate, skin, breast, bladder, bowel and colon cancer).

FIGURE 2

Cause of death in patients with idiopathic pulmonary fibrosis (IPF). Data presented are the reported cause of death (n, % of patients) up to 31 October 2019. 20 separate causes of death were listed under “other” including a number of cancers (lung, bladder, prostate and stomach cancer), multi-organ failure and sepsis. In 30 cases, the cause of death was not known.