Abstract
Background: Idiopathic Pulmonary Fibrosis (IPF) is a debilitating lung disease with average life expectancy of 3-5 years. IPF registries are key to our understanding of disease presentation and behaviour in the real-world setting.
Aim: Analysis of Manchester Foundation Trust IPF registry patient demographics, clinical work-up and co-morbidities.
Methods: We are a specialist interstitial lung disease service in the North West of England and the largest contributor to the British Thoracic Society (BTS) IPF Registry.
Results: We have 451 of 1119 (41%) patient records on the BTS-IPF registry. 350 (78%) are male with a median presentation age of 71.7 years (range 47-90). At presentation, 78% of patients were symptomatic >12 months of which 59% are over 2 years. At presentation 81% of patients have comorbidities, the commonest being hypertension (30%), ischaemic heart disease (23%), diabetes (20%) and gastroesophageal reflux (10%). 32% of patients were GAP stage 1, 55% stage 2 and 14% were stage 3. 39% have definite UIP pattern on HRCT, 55% have possible UIP and 6% are inconsistent. Despite this only 12% have a biopsy diagnosis. At review 35% of patients have a forced vital capacity greater than 80%, which is outside the UK antifibrotic prescription criteria with an average transfer factor of 44%. Overall 69% patients are prescribed antifibrotic therapy.
Conclusion: Our patients present years after becoming symptomatic, with significant lung function impairment and co-morbidities. 69% of our patients are prescribed antifibrotics which is higher than published uptake in Europe and the US. Our registry provides insights into the diagnosis and management of IPF patients in the real-world setting.
Aim: Analysis of Manchester Foundation Trust IPF registry patient demographics, clinical work-up and co-morbidities.
Methods: We are a specialist interstitial lung disease service in the North West of England and the largest contributor to the British Thoracic Society (BTS) IPF Registry.
Results: We have 451 of 1119 (41%) patient records on the BTS-IPF registry. 350 (78%) are male with a median presentation age of 71.7 years (range 47-90). At presentation, 78% of patients were symptomatic >12 months of which 59% are over 2 years. At presentation 81% of patients have comorbidities, the commonest being hypertension (30%), ischaemic heart disease (23%), diabetes (20%) and gastroesophageal reflux (10%). 32% of patients were GAP stage 1, 55% stage 2 and 14% were stage 3. 39% have definite UIP pattern on HRCT, 55% have possible UIP and 6% are inconsistent. Despite this only 12% have a biopsy diagnosis. At review 35% of patients have a forced vital capacity greater than 80%, which is outside the UK antifibrotic prescription criteria with an average transfer factor of 44%. Overall 69% patients are prescribed antifibrotic therapy.
Conclusion: Our patients present years after becoming symptomatic, with significant lung function impairment and co-morbidities. 69% of our patients are prescribed antifibrotics which is higher than published uptake in Europe and the US. Our registry provides insights into the diagnosis and management of IPF patients in the real-world setting.
| Original language | English |
|---|---|
| Article number | PA2195 |
| Journal | European Respiratory Journal |
| DOIs | |
| Publication status | Published online - 19 Nov 2018 |
