S97 Is an Increased Tendency to Clot a Risk Factor For Developing Idiopathic Pulmonary Fibrosis?

Abstract

Background The aetiology of idiopathic pulmonary fibrosis (IPF) remains poorly understood. Recent animal studies and epidemiological data have suggested that activation of the coagulation cascade in the lung may have an important role in the pathogenesis of IPF. Methods We recruited incident cases of physician diagnosed IPF from five teaching hospitals and eight district general hospitals in England and Wales. Age and sex matched general population controls were recruited from the same region. Participants were asked for details of lifetime occupational history, current or previous illnesses, medication and smoking. Each case and control then had a venous blood sample taken for a thrombophilia screen, including inherited and acquired clotting defects. We also collected high resolution computed tomography (HRCT) scans for all cases, which were reviewed by two experienced thoracic radiologists to confirm

Risk Factors for Cardiovascular Disease in People With Idiopathic Pulmonary Fibrosis

“…10 Furthermore, as IPF is usually diagnosed in secondary care, it seems unlikely that a record of this diagnosis will be found in primary care records without confirmation from secondary care. The age distribution of cases with IPF in this study is similar to other population based cohorts of IPF, [12][13][14][15][16] providing reassurance of the validity of diagnosis of IPF in these data. In addition, a study by Fell and colleagues demonstrated that age is a strong predictor of IPF in patients with an atypical Usual Interstitial Pneumonia (UIP) radiographic pattern, whereby individuals over 65 years had 89% specificity for confirming IPF on lung biopsy.…”

“…It is possible, however, that IPF and ischaemic heart disease share other aetiological risk factors. Studies have suggested that people with IPF have an increased tendency to clot, 14,27,28 and have higher Factor VIII levels than the general population. 14 Previous studies have also demonstrated that elevated Factor VIII levels are associated with an increased risk of coronary artery disease.…”

“…Studies have suggested that people with IPF have an increased tendency to clot, 14,27,28 and have higher Factor VIII levels than the general population. 14 Previous studies have also demonstrated that elevated Factor VIII levels are associated with an increased risk of coronary artery disease. 29,30 In conclusion, we have found that people with IPF have increased prevalence of cardiovascular risk factors.…”

Venous thromboembolism in people with idiopathic pulmonary fibrosis: a population-based study

“…Possible explanations for our finding include 1) IPF increasing the risk of VTE, and 2) a prothrombotic state leading to the development of IPF and VTE. This study supports the hypothesis that activation of the coagulation cascade may be involved in the pathogenesis of IPF [2,7]. …”

Surgery for secondary pneumothorax caused by Hermansky–Pudlak syndrome

“…Possible explanations for our finding include 1) IPF increasing the risk of VTE, and 2) a prothrombotic state leading to the development of IPF and VTE. This study supports the hypothesis that activation of the coagulation cascade may be involved in the pathogenesis of IPF [2,7]. @ERSpublications People with IPF have higher incidence rates of venous thromboembolism and are more likely to be prescribed warfarin…”