| 1. Significant new evidence has been made available in IPF since the recent international guidelines published in 2011 |
| 2. In a 12-month, phase II, double-blind, placebo-controlled randomised trial of patients with IPF, the triple tyrosine kinase inhibitor BIBF1120 (nintedanib) 150 mg twice daily was associated with a 68.4% reduction in the rate of decline in FVC (p=0.06) |
| 3. The triple therapy combining prednisone, azathioprine and N-acetylcysteine was associated with an increased rate of all-cause mortality and hospitalisation compared to placebo, providing compelling evidence against the initiation of this combination in patients with IPF |
| 4. Results of clinical trials in IPF indicated that anticoagulation therapy and endothelin receptor antagonists are either harmful (especially ambrisentan) or ineffective and are not recommended as treatment for IPF |
| 5. Delayed access to a tertiary care centre is associated with a higher risk of death in patients with IPF independent of disease severity |
| 6. Concomitant emphysema, DLCO <47% of predicted value, and pulmonary hypertension are independent predictors of acute exacerbation of IPF |
| 7. Standardised oxygen requirements, pulmonary hypertension at baseline and acute exacerbations of IPF predict mortality in patients with IPF |
| 8. A simple-to-use staging system (“GAP” score) based on sex, age, FVC (% predicted), and DLCO (% predicted), predicts the individual risk of 1-, 2- and 3-yr mortality, and may be helpful for management decisions in patients with IPF |
| 9. In a population of systemic sclerosis patients, the alveolar nitric oxide concentration accurately identifies patients with a high risk of developing lung function deterioration or death. |
| 10. Patients with connective tissue disease and interstitial lung disease have a better long-term prognosis than patients with IPF |
| 11. In the setting of interstitial lung disease, undifferentiated connective tissue disease (also referred to as autoimmune-featured interstitial lung disease or lung-dominant connective tissue disease), defined by symptoms and/or biological autoimmune features without diagnostic criteria for a given autoimmune disease, is associated with a higher frequency of nonspecific interstitial pneumonia pattern, female sex, age <50 yrs, and Raynaud's phenomenon compared to idiopathic counterparts |
| 12. Patients with sarcoidosis diagnosed after the age of 65 yrs are more frequently female, more frequently have asthenia, uveitis, specific skin lesions and corticosteroid-related adverse events, and less frequently have erythema nodosum than younger patients |
IPF: idiopathic pulmonary fibrosis; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide.