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Background:  Interstitial lung disease (ILD) is a common complication in patients with systemic sclerosis (SSc), and its diagnosis contributes to early treatment decisions.   Purposes:  To quantify ILD associated with SSc (SSc-ILD) from chest CT images using an automatic quantification method based on the computation of the weight of interstitial lung opacities.   Methods:  Ninety-four patients with SSc underwent CT, forced vital capacity (FVC), and carbon monoxide diffusion capacity (DL CO ) tests. Seventy-three healthy individuals without radiological evidence of lung disease served as controls. After lung and airway segmentation, the ratio between the weight of interstitial opacities [densities between −500 and +50 Hounsfield units (HU)] and the total lung weight (densities between −1,000 and +50 HU) was used as an ILD indicator (ILD[%] = 100 × [LW (−500 to +50HU) /LW (−1, 000 to +50HU) ]). The cutoff of normality between controls and SSc was determined with a receiver operator characteristic curve. The severity of pulmonary involvement in SSc patients was also assessed by calculating  Z  scores of ILD relative to the average interstitial opacities in controls. Accordingly, SSc-ILD was classified as SSc Limited-ILD ( Z  score < 3) and SSc Extensive-ILD ( Z  score ≥ 3 or FVC < 70%).   Results:  Seventy-eight (83%) SSc patients were classified as presenting SSc-ILD (optimal ILD threshold of 23.4%, 0.83 sensitivity, 0.92 specificity, and 0.94 area under the receiver operator characteristic curve, 95% CI from 0.89 to 0.96, 0.93 positive predictive value, and 0.81 negative predictive value,  p  < 0.001) and exhibited radiological attenuations compatible with interstitial pneumonia dispersed in the lung parenchyma. Thirty-six (38%) patients were classified as SSc Extensive-ILD (ILD threshold ≥ 29.6% equivalent to a Z score ≥ 3) and 42 (45%) as SSc Limited-ILD. Eighteen (50%) patients with SSc Extensive-ILD presented FVC < 70%, being only five patients classified exclusively based on FVC. SSc Extensive-ILD also presented lower DL CO  (57.9 ± 17.9% vs. 73.7 ± 19.8%;  p  < 0.001) and total lung volume (2,916 ± 674 vs. 4,286 ± 1,136,  p  < 0.001) compared with SSc Limited-ILD.   Conclusion:  The proposed method seems to provide an alternative to identify and quantify the extension of ILD in patients with SSc, mitigating the subjectivity of semiquantitative analyzes based on visual scores.

Automatic Quantification of Interstitial Lung Disease From Chest Computed Tomography in Systemic Sclerosis