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Diagnosis of interstitial lung disease (ILD) secondary to systemic sclerosis (SSc) and rheumatoid arthritis (RA) and identification of ‘progressive pulmonary fibrosis’ using chest CT: a narrative review

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A Correction to this article was published on 20 November 2023

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Abstract

Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs), with incidence and prevalence variously assessed in the literature but reported in up to 30% of patients, with higher frequency in rheumatoid arthritis (RA) and systemic sclerosis (SSc). Recent years have seen a growing interest in the pulmonary manifestations of ILD-CTDs, mainly due to the widening of the use of anti-fibrotic drugs initially introduced exclusively for IPF, and radiologists play a key role because the lung biopsy is very rarely used in these patients where the morphological assessment is essentially left to imaging and especially HRCT. In this narrative review we will discuss, from the radiologist’s point of view, the most recent findings in the field of ILD secondary to SSc and RA, with a special focus about the progression of disease and in particular about the ‘progressive pulmonary fibrosis’ (PPF) phenotype, and we will try to address two main issues: How to predict a possible evolution and therefore a worse prognosis when diagnosing a new case of ILD-CTDs and how to assess the progression of an already diagnosed ILD-CTDs.

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The authors have not disclosed any funding. All the authors declare that they have no competing interests and didn't receive a specific grant for this research.

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Authors and Affiliations

  1. Radiology Unit - S. Maria della Scaletta Hospital, Imola, Italy

    Antonio Poerio

  2. Radiology Unit - Niguarda Hospital, Milan, Italy

    Eleonora Carlicchi

  3. Department of Radiology - Villa Erbosa, Gruppo San Donato, Bologna, Italy

    Maurizio Zompatori

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  1. Antonio Poerio
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  2. Eleonora Carlicchi
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  3. Maurizio Zompatori
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Conception and design were analysed by MZ. Collection and interpretation of data were performed by AP and EC. Drafting of the manuscript was conducted by AP, EC and MZ. Final review of the manuscript was provided by MZ. All authors have read and approved the final manuscript.

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Correspondence to Antonio Poerio.

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Poerio, A., Carlicchi, E. & Zompatori, M. Diagnosis of interstitial lung disease (ILD) secondary to systemic sclerosis (SSc) and rheumatoid arthritis (RA) and identification of ‘progressive pulmonary fibrosis’ using chest CT: a narrative review. Clin Exp Med 23, 4721–4728 (2023). https://doi.org/10.1007/s10238-023-01202-1

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