Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history — sgalla 2015

The diagnosis of idiopathic pulmonary fibrosis (IPF) requires rigorous exclusion of secondary causes for pulmonary fibrosis and the presence of a pattern of typical interstitial pneumonia (UIP) at either a surgical lung biopsy or a high-resolution computed tomography (HRCT) scan. IPF diagnosis remains difficult, especially in those who present with aberrant HRCT patterns, despite significant efforts to create precise, widely accepted diagnostic criteria. Establishing a correct diagnosis is turning into a therapeutically relevant issue as new medications are introduced. IPF epidemiology is debatable despite the fact that it is a rare disease since research have relied on outdated data and used inconsistent, heterogeneous approaches to define patients. Overall, it appears that the prevalence and incidence have been rising over the past few decades, which raises the possibility that prior research may have underestimated them due to diagnostic ambiguity. Although the clinical course of IPF can widely vary from person to person and periods of severe acute respiratory deterioration (acute exacerbations) are unpredictable, IPF is always progressing. A better classification of the disease—essential for providing customized therapy approaches—would result from a fuller knowledge of the mechanisms underlying an accelerated course of the disease and the discovery of biomarkers of progression.

Sgalla G, Biffi A, Richeldi L. Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history. Respirology. 2016 Apr;21(3):427-37. doi: 10.1111/resp.12683. Epub 2015 Nov 23. PMID: 26595062.