Quantitative HRCT in Interstitial Lung Disease Secondary to Systemic Sclerosis: Evaluation of Disease Progression and Therapeutic Response
Author Block: V. Uggenti1, M. Di Battista1, A. Della Rossa1, L. Tavanti1, A. De Liperi1, B. Bartholmai2, M. Mosca1, E. Neri1, C. Romei1; 1Pisa/IT, 2Rochester, MN/US
Purpose: This study aimed to assess, using CALIPER-derived quantitative parameters, the longitudinal course of interstitial lung disease secondary to systemic sclerosis (SSc-ILD), a complex condition characterized by alternating inflammatory exacerbations and progressive fibrotic changes.
Methods or Background: 50 SSc-ILD patients were included in the study (36 females; 14 males), with a mean age at baseline HRCT of 57.1 ± 13.0 years and a mean disease duration of 60.9 ± 81.5 months. Among them, 43 (86%) were receiving immunosuppressive therapy, and 7 (14%) were receiving antifibrotic treatment. All patients underwent at least two follow-up HRCT scans. All HRCTs were processed using CALIPER, which performs lung texture analysis, providing percentages of honeycombing (HC), reticulations (RET), ground glass (GG), vascular-related structures (VRS), and normal lung. ILD% was defined as the sum of GG%, RET%, and HC%.
Results or Findings: Pulmonary arterial hypertension (PAH) was associated with higher ILD (p=0.006) and VRS (p<0.001) values.
Among patients receiving immunosuppressive therapy, RET+HC showed a progressive increase over time. Conversely, GG displayed a distinct trend: in patients with baseline GG% <20%, follow-up oscillations suggested higher responsiveness to treatment; in contrast, patients with extensive baseline involvement (>20%) exhibited a progressive increase in GG.
ILD% was an independent predictor of pulmonary function: higher ILD% was associated with reduced FVC (β = –0.50, SE = 0.12, p<0.001) and DLCO (β = –0.77, SE = 0.11, p<0.001).
Conclusion: Higher ILD% and VRS% scores correlate with PAH.
The subanalysis of individual interstitial alterations suggests that GG% oscillations reflect responsiveness to immunosuppressive therapy.
The inverse correlation between ILD% and both FVC and DLCO supports the prognostic value of quantitative parameters.
Quantitative HRCT may support tailored therapeutic management in SSc-ILD.
Limitations: Single-center, retrospective design, with potential selection bias.
Imbalanced treatment groups.
Heterogeneous follow-up.
Funding for this study: None.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: Interstiziopatia polmonare in corso di malattie autoimmuni sistemiche: correlazione del fenotipo polmonare con caratteristiche cliniche e sierologiche, uno studio osservazionale (AISILD); Azienda Ospedaliero-Universitaria Pisana, Via Roma 67, Pisa; UO Reumatologia AOUP; Protocollo versione 2 del 06/04/2024.