Francesco Amati

Background: Accurate radiological characterization of interstitial lung disease (ILD) is critical for multidisciplinary discussion (MDD) and treatment decisions. Photon-counting computed tomography (PC-CT) provides higher spatial resolution than conventional high-resolution CT (HRCT), potentially improving fibrotic pattern assessment. Aims: To determine whether PC-CT modifies guideline-based radiological pattern classification and diagnostic confidence compared with HRCT in ILD, and to assess its impact on multidisciplinary diagnosis. Methods: In this ongoing prospective single-centre study (Humanitas Research Hospital, Rozzano, Milan), consecutive ILD patients undergo same-day HRCT and PC-CT. Scans are independently evaluated by two expert thoracic radiologists according to current guideline criteria. Patterns are classified as Usual Interstitial Pneumonia (UIP), probable UIP, indeterminate for UIP, or alternative diagnosis. Diagnostic confidence is recorded using a standardized framework. The primary endpoint is radiological pattern reclassification between HRCT and PC-CT. Secondary endpoints include changes in diagnostic confidence and multidisciplinary diagnosis. Results: To date, 28 ILD patients have been enrolled. PC-CT led to radiological reclassification in 9/28 patients (32%). Among those classified as indeterminate for UIP on HRCT (n=7), 4 (57%) were reclassified as probable UIP on PC-CT. Multidisciplinary reassessment resulted in management changes in 2 patients (7%), including new diagnoses of idiopathic pulmonary fibrosis. Conclusions: Our preliminary results suggest that PC-CT is associated with increased diagnostic confidence and pattern recognition in ILD, with potential impact on multidisciplinary diagnosis. Further enrolment is ongoing.

In sarcoidosis, personalised strategies that consider the various phenotypes and endotypes of the disease have the potential to improve patient care. The ‘treatable traits’ (TT) approach offers a promising strategy that has already been proposed and implemented in several other chronic pulmonary diseases. In this review, we propose five distinct TT categories for patients with sarcoidosis: aetiological, lifestyle, pulmonary, extrapulmonary (systemic) and comorbidities. Despite the potential of this strategy, several challenges hinder its implementation. In addition, much of the knowledge on the definition and prevalence of TTs comes from single-centre studies. Creating international registries will enhance our understanding and provide more accurate data on these traits. TTs are dynamic and require continuous evaluation to effectively respond to changes caused by disease progression or treatment response. A multidisciplinary management approach is therefore crucial as sarcoidosis can impact multiple organs. Integrating primary care physicians into the management framework will improve overall patient care. Incorporating patient preferences into the TT strategy is vital for improving adherence to treatment. Additionally, using patient-reported outcome measures will provide valuable insights into the impact of treatment. While the TT approach is feasible in developed healthcare systems, challenges exist in resource-limited settings. Advancing patient-centred care towards 5P medicine involves addressing key TTs that meet the unique patient needs. Therefore, randomised controlled trials focused on TTs are needed. Our proposed TT strategy aims to enhance quality of life and outcomes for patients with sarcoidosis while increasing awareness of the importance of TTs among healthcare providers and advocacy groups.

Progressive interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Early recognition of progression is crucial to timely treatment, but reliable early markers are lacking. Automated quantitative analysis of chest high-resolution computed tomography (HRCT) may provide an objective tool to detect early progression. This study aims to compare automated and visual assessment in identifying early SSc-ILD progression and evaluate their predictive value for subsequent functional decline. In this retrospective longitudinal study, 33 patients with SSc-ILD underwent HRCT and pulmonary function tests (PFTs) at baseline (T1) and after one year (T2; IQR 0.75-2.5 years). PFTs were repeated 12 months after T2 (T3; IQR 9-14.5 months, T3). Two radiologists performed blinded semi-quantitative visual scoring of fibrosis and ground-glass opacity (GGO), while automated analysis was conducted using Thoracic VCAR (GE Healthcare). Associations with PFTs and prediction of functional progression (Erice/INBUILD criteria) and treatment escalation were analyzed. Both visual and automated analyses detected fibrosis progression (visual: 1.5 → 1.6, p= 0.0005; software: 0.34%→0.4%, p= 0.01). Only automated analyses identified increases in GGO (15.5%→15.8%, p= 0.04) and reduced normal lung tissue (75.6%→73%, p= 0.009). Software-derived parameters correlated strongly with PFT changes, whereas visual scores showed weaker correlations. Early (T1-T2) automatically-detected GGO increases predicted subsequent functional decline at T3 (p= 0.04), with changes > 50 mL (8.2%) yelding 81% sensitivity and 77% specificity. Automated quantitative HRCT analysis, but not visual evaluation, predicts long-term functional progression in SSc-ILD.

Background: Rehabilitation represents a key nonpharmacological treatment for patients with interstitial lung diseases (ILDs). As a consequence of improvement in technologies and social distancing in the SARS-CoV-2 era, a growing body of evidence demonstrated the effectiveness of tele-rehabilitation programmes for chronic respiratory diseases. Methods: We conducted a systematic review to identify randomised controlled trials (RCTs) on tele-rehabilitation in ILD and extended the search to ongoing trials. Results: Throughout our research, we identified four RCTs describing home rehabilitation in ILD. All RCTs were heterogenous small trials. Thus, a meta-analysis could not be performed, and data were reported in a descriptive way. The primary outcomes of the RCTs analysed included both physical and functional measures. We also identified through ClinicalTrials.gov four ongoing clinical trials investigating pulmonary rehabilitation in ILD with a considerable degree of heterogeneity regarding the populations included. In our systematic review, we also highlighted the key pillars for future trials in the field. Conclusion: Potential treatment trajectories on outcomes of ILD patients related to the implementation of tele-rehabilitation are also discussed.

The classification of interstitial lung disease (ILD)s has traditionally relied on well-defined diagnostic labels, such as idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), and hypersensitivity pneumonitis (HP)

Interstitial lung diseases (ILD) comprise a heterogeneous group of disorders that present significant challenges in both diagnosis and management due to their varied nature, which encompasses different degrees of inflammation and fibrosis affecting lung tissue

Background: The 2018 guidelines on the diagnosis of idiopathic pulmonary fibrosis (IPF) conditionally recommend multidisciplinary discussion (MDD) for diagnostic decision-making. However, limited data concerning the diagnostic impact of MDD on interstitial lung diseases (ILDs) are available. Objectives: The objective of this prospective study was to assess the impact of MDD at a tertiary referral ILD center on diagnostic trajectories, prognosis, and identification of potential treatable traits in ILD management. Design: This prospective study enrolled all consecutive adult ILD patients referred for MDD to a tertiary academic center in San Antonio, TX, USA from January 2017 to May 2020. The subjects were followed during a 3-year follow-up period after the MDD. Methods: Patients were stratified into three groups according to the pre-MDD diagnosis: unspecified ILD, IPF, and not IPF, and compared to the re-stratification post-MDD diagnosis into: unclassifiable ILD, IPF, and not IPF. The primary outcome was the percentage change in diagnostic trajectories after the MDD discussion. Results: A total of 201 ILD patients (61.7% male; mean (DS) age: 67.2 (10.4) years) were included in the study. The total diagnostic trajectory change occurred in 122 (60.7%) patients. The diagnostic trajectories changed in 40 (46.5%) patients in the IPF group and 8 (19.5%) in the non-IPF group ( p -value = 0.0003). Patients with pre-MDD unspecified-ILD were classified as not-IPF in 32.4% ( n = 24), IPF in 23% ( n = 17), and unclassifiable-ILD in 44.6% ( n = 33) post-MDD. Considering the post-MDD diagnosis, differences in mortality were detected among the three groups ( p = 0.037). Conclusion: Our results suggest that MDD has a significant impact not only on the diagnostic trajectories (DT) but also on the prognosis of patients with ILDs.

Current evidence shows that the diagnostic accuracy for interstitial lung diseases (ILD) increased through the multidisciplinary discussion (MDD). However, a significant heterogeneity among ILD centers exists in terms of MDD composition and few data on direct comparison of MDD have been published so far. To address this clinical challenge, data from MDDs of two ILD referral centers have been compared. Patients discussed during MDD were prospectively recruited in two referral ILD centers (San Antonio, TX, USA, and Rozzano, Milan, Italy). The same case report form (CRF) was used in both centers. Pre-MDD and post-MDD diagnosis were assessed for all the patients. Pre-MDD diagnoses were categorized into three groups, idiopathic pulmonary fibrosis (IPF), not-IPF, and unspecified ILD, while post-MDD diagnoses were IPF, not-IPF, and unclassifiable ILD. The primary objective of the study was the absolute change in diagnostic trajectories post-MDD across the two centers. 242 patients (median age: 69 years; 56% males) were included. 62% of diagnoses (n=124) were changed following MDD in San Antonio versus 17% (n=7) in Rozzano (p<0.001). Treatment was changed post-MDD in 53% (n=106) of ILD patients in San Antonio and 34% (n=14) in Rozzano (p=0.039). Additional tests and/or specialistic referral were required in 63% (n=127) of ILD patients post-MDD in San Antonio versus 78% (n=32) in Rozzano (p=0.073). Our study strengthens the knowledge that MDD has an impact on diagnostic trajectories and management of ILD patients.