Lancet
-
Some of the most pressing challenges associated with interstitial lung disease (ILD) are how best to define, diagnose, and treat connective tissue disease-associated ILD (CTD-ILD)--disorders with potentially substantial morbidity and mortality. In this focused review, we address aspects of prognosis for CTD-ILD and what indices might predict outcome, together with lessons that can be learnt from clinical trials of systemic sclerosis-associated ILD and idiopathic pulmonary fibrosis and how these lessons might be applied to future studies of CTD-ILD.
-
Idiopathic pulmonary fibrosis is a serious and progressive chronic lung disease that is characterised by altered cellular composition and homoeostasis in the peripheral lung, leading to excessive accumulation of extracellular matrix and, ultimately, loss of lung function. It is the interstitial pneumonia with the worst prognosis--mortality 3-5 years after diagnosis is 50%. During the past decade, researchers have described several novel cellular and molecular mechanisms and signalling pathways implicated in the pathogenesis of idiopathic pulmonary fibrosis, resulting in the identification of new therapeutic targets. These advances will hopefully result in increased survival rates and improved quality of life for patients with this disorder in future.