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Idiopathic pulmonary fibrosis (IPF) is a fatal condition with limited treatment options. The diagnosis is usually made radiologically but a careful history to exclude identifiable causes of interstitial lung disease is required. Although the median survival is poor, there is considerable variability in disease progression. This thesis investigates the diagnostic accuracy of IPF, a novel treatment option for IPF and biomarkers predictive of disease progression. An audit project determined a confident diagnosis of IPF in only 9% of patients due to lack of aetiological factors assessment. In a double-blind, multi-centre study, 181 patients with fibrotic idiopathic interstitial pneumonia was randomised to receive co-trimoxazole or placebo for 12 months in addition to usual care. Measurements were made of forced vitalcapacity (FVC), and quality adjusted life years (QALYs). All-cause mortality, costs, and adverse events were recorded. Co-trimoxazole had no effect on measures of lung function. However in those adhering to treatment, co-trimoxazole showed a significant reduction in all-cause mortality associated with reduction in respiratory tract infections and improvements in QALYs gained. Treatment with co-trimoxazole was cost-effective at UK thresholds. The role of clinical-physiological parameters, exhaled alveolar nitric oxide concentration (CaNO) and plasma vascular endothelial growth factor (VGEF) in predicting disease behaviour were assessed in two studies. Prednisolone therapy was predictive of death at 12 months and 10% decline in FVC at 6 months, St George’s Respiratory Questionnaire score was predictive of death at 12 months. A prospective pilot study of 27 patients with IPF showed that CaNO has a strong predictive value for subsequent significant decline in diffusing capacity of carbon monoxide and that VGEF level is a strong predictive factor of subsequent significant FVC decline. Structured proforma could aid diagnostic process in IPF while biomarkers could be used in prediction models. Survival of IPF is improved by prophylactic treatment with antibiotics.