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Review
. 2021 Jan;478(1):89-99.
doi: 10.1007/s00428-020-02964-9. Epub 2020 Nov 9.

Molecular approach to the classification of chronic fibrosing lung disease-there and back again

Stijn E Verleden  1   2 , Peter Braubach  3   4 , Mark Kuehnel  3   4 , Nicolas Dickgreber  5 , Emily Brouwer  3   4 , Pauline Tittmann  3   4 , Florian Laenger  3   4 , Danny Jonigk  3   4
Affiliations
Review

Molecular approach to the classification of chronic fibrosing lung disease-there and back again

Stijn E Verleden et al. Virchows Arch. 2021 Jan.

Abstract

Chronic diffuse parenchymal lung disease (DPLD) is an umbrella term for a very heterogeneous group of lung diseases. Over the last decades, clinical, radiological and histopathological criteria have been established to define and separate these entities. More recently the clinical utility of this approach has been challenged as a unifying concept of pathophysiological mechanisms and a shared response to therapy across the disease spectrum have been described. In this review, we discuss molecular motifs for subtyping and the prediction of prognosis focusing on genetics and markers found in the blood, lavage and tissue. As a purely molecular classification so far lacks sufficient sensitivity and specificity for subtyping, it is not routinely used and not implemented in international guidelines. However, a better molecular characterization of lung disease with a more precise identification of patients with, for example, a risk for rapid disease progression would facilitate more accurate treatment decisions and hopefully contribute to better patients' outcomes.

Keywords: Biomarker; Diffuse parenchymal lung disease; Idiopathic pulmonary fibrosis; Interstitial lung disease; Lung fibrosis.

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