The continuing struggle in the ﬁeld of interstitial lung diseases (ILDs) has long been how best to classify disease on the basis of etiology or shared pathogenetic mechanisms. In the era of antiﬁbrotic therapeutics of proven efﬁcacy, accurate diagnosis is more important than ever. The articles published in 2018 reﬂect this ongoing debate in the medical community. The year 2018 led to new recommendations for the diagnosis of idiopathic pulmonary ﬁbrosis (IPF) with active efforts to do the same for chronic hypersensitivity pneumonitis (CHP). Over the past two decades, international agreement on diagnostic criteria of IPF allowed a deeper understanding of disease mechanisms to be reached and facilitated the achievement of outstanding therapeutic goals. However, continual reclassiﬁcation should not restrain efforts aimed at the discovery of therapies that can be beneﬁcial for patients with diverse forms of progressive ﬁbrotic disease, who so desperately need interventions that improve both quality of life and lifespan. With an exciting array of new therapeutic targets emerging from basic laboratory investigations, the responsibility of balancing careful phenotyping while addressing compelling clinical needs via efﬁcient trial design will rest on the respiratory community. Additional work highlights the need for pulmonologists to be advocates for the protection and support of their patients. Within the scope of this paper, we review the latest advances in diagnosis, management, and pathogenesis of IPF, which is still the focus of most of the published research in the ﬁeld of ILD. We then revise the most relevant evidence published on non-IPF ﬁbrotic lung disease, acknowledging that in the future more attention should be focused on ﬁnding efﬁcacious treatments for those patients with progressive disease, regardless of speciﬁc etiologies and strict classiﬁcation schemes.
|Numero di pagine||9|
|Rivista||American Journal of Respiratory and Critical Care Medicine|
|Stato di pubblicazione||Pubblicato - 2019|
- Pulmonary Fibrosis