REVIEW  HRCT OF THE CHEST IN ILD 

Journal of Radiological Review 2021 June;8(2):121-31

DOI: 10.23736/S2723-9284.21.00134-0

Copyright © 2021 EDIZIONI MINERVA MEDICA

language: English

Focus on progressive fibrosing interstitial lung diseases (PF-ILDs)

Giuseppe CICCHETTI ^{1, 2} ✉, Annemilia DEL CIELLO ¹, Lucio CALANDRIELLO ¹, Elisa LAROSA ^{1, 2}, Paola FRANCHI ³, Alessandra FARCHIONE ¹, Manuela MEREU ⁴, Roberto IEZZI ^{1, 2}, Stefano PALMUCCI ⁵, Anna Rita LARICI ^{1, 2}

¹ Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, “A. Gemelli” University Polyclinic Foundation IRCCS, Rome, Italy; ² Department of Radiological and Hematological Sciences, Section of Radiology, Catholic University of the Sacred Heart of Rome, Rome, Italy; ³ Department of Diagnostic Radiology, “G. Mazzini” Hospital, Teramo, Italy; ⁴ Department of Radiology, Santissima Annunziata Hospital, Chieti, Italy; ⁵ Department of Medical Surgical Sciences and Advanced Technologies “G.F. Ingrassia” Unit of Radiology I, Polyclinic “G. Rodolico-San Marco” University Hospital, University of Catania, Catania, Italy

The term progressive fibrosing interstitial lung diseases (PF-ILDs) encompass a subset of ILD patients experiencing progressive lung function decline, reduced physical performance, and quality of life, mostly associated with increased extent of fibrotic abnormalities on high-resolution computed tomography (HRCT) scans, despite adequate treatment. Although idiopathic pulmonary fibrosis (IPF) is considered the prototype of progressive fibrotic diseases, the concept of PF-ILD can be applied to a variety of ILDs, which show some similarities in morphology, underlying pathologic mechanisms, and risk factors with IPF. Recently, evidences have emerged to support the role of antifibrotic therapy in slowing the disease progression, regardless the underlying ILD type, opening up new frontiers in the diagnostic and therapeutic management of these patients. This review article represents a focus on the current knowledge of PF-ILDs and aims to point out the role of imaging and emerging biomarkers in the assessment of disease progression and patient stratification. The most common PF-ILDs other than IPF will be described, highlighting their known risk factors for progression and reduced survival. Finally, the new perspectives in the management and research of this evolving field of pulmonary medicine will be briefly described.

KEY WORDS: Interstitial lung disease; Progressive fibrosing interstitial lung disease; Pulmonary fibrosis; High-resolution computed tomography; Antifibrotic therapy