Historical milestones in the understanding and management of idiopathic pulmonary fibrosis (IPF)

1935Description of “fulminating diffuse interstitial fibrosis of the lungs” by L. Hamman and A.R. Rich[89]
1968First classification of interstitial pneumonias, A.A. Liebow and D. E. Smith, including usual or classical interstitial pneumonia (UIP)[90]
1978Individualisation of idiopathic pulmonary fibrosis (IPF) (or cryptogenic fibrosing alveolitis)[91]
1981–1985Individualisation of organising pneumonia[94]
1984IPF, an inflammatory disease of alveolar macrophage[95]
1989–1991Giant cell interstitial pneumonia (GIP) is related to exposure to hard metals and is not idiopathic[96, 97]
1994Description of non-specific interstitial pneumonia (NSIP)[98]
2000International consensus on IPF, defined by a UIP pattern at HRCT and/or biopsy, in idiopathic setting[99]
2001IPF, a disease related to epithelial cell dysfunction and myofibroblast dysfunction[8]
2002International ATS/ERS/JRS/ALAT classification of idiopathic interstitial pneumonias (IIPs), including NSIP[1]
2002Description of acute fibrinous organising pneumonia (AFOP)[100]
2005Individualisation of combined pulmonary fibrosis and emphysema syndrome (CPFE)[101]
2007Description of familial pulmonary fibrosis associated with telomerase complex mutations[102]
2011International ATS/ERS/JRS/ALAT guidelines on diagnosis and management of IPF[3]
2011IPF is associated with a common polymorphism in the promoter of MUC5B[7]
2013Update of international ATS/ERS/JRS/ALAT classification of IIPs, including pleuroparenchymal fibroelastosis, AFOP, unclassifiable IIPs and a disease behaviour classification[19]
2014Demonstration of efficacy of pirfenidone and nintedanib in IPF[38, 103]
2015Update of ATS/ERS/JRS/ALAT treatment guidelines on IPF[104]
2017Expert group endorsement of multidisciplinary diagnosis of IPF in patients not meeting formal guideline diagnostic criteria[105]