Data indicative of likely shared pathogenetic mechanisms between idiopathic pulmonary fibrosis (IPF) and other progressive fibrotic lung diseases; and subgroups of patients with diseases other than IPF with a progressive IPF-like fibrotic phenotype
| Reference | |
| Data indicative of IPF-like disease progresssion in subgroups of patients with other progressive fibrotic lung diseases | |
| IPF-like outcomes in CHP patients with a histological or CT pattern of UIP | [46–51] |
| IPF-like outcomes in RA patients with a histological or CT pattern indicative of UIP | [51–54, 58–61] |
| IPF-like outcomes in IPAF patients with a histological or CT pattern indiciative of UIP | [63] |
| Outcomes intermediate between IPF and other progressive fibrotic diseases in patients with unclassifiable ILD | [65–67] |
| Reports of patients with drug-induced lung disease exhibiting a fatal progressive fibrotic phenotype despite drug withdrawal | [70] |
| IPF-like outcomes in patients with idiopathic NSIP with disease progression at 6–12 months (as judged by serial FVC trends) | [68, 69] |
| Linkage between serial decline in FVC and mortality in CHP, SSc-ILD and rheumatoid lung, similar to that seen in IPF | [59, 72, 73] |
| Data indicative of pathogenetic mechanisms common to IPF and other progressive fibrotic lung diseases | |
| Shared genetic predilection for IPF and rheumatoid lung | [76] |
| Similar links between short telomere lengths and mortality in IPF and CHP | [77, 78] |
| Linkage between alveolar epithelial cell dysfunction/injury and pulmonary function decline in IPF and SSc-ILD | [79] |
| Pathobiological mechanisms likely to contribute to disease progression in both IPF and SSc-ILD: alveolar stem cell exhaustion/cellular senescence, mitochondrial dysfunction, impaired autophagy, epigenetic modifications, and immune dysregulation | [80–83] |
CHP: chronic hypersensitivity pneumonitis; CT: computed tomography; UIP: usual interstitial pueumonia; RA: rheumatoid arthritis; IPAF: interstitial pneumonitis with autoimmune features; ILD: interstitial lung disease; NSIP: non-specific interstitial pneumonia; FVC: forced vital capacity; SSc: systemic sclerosis.