Table 1. Twelve key messages
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.