High-Resolution Computed Tomography: Lights and Shadows in Improving Care for SSc-ILD Patients

Abstract

The diagnosis and classification of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is essential to improve the prognosis of systemic sclerosis (SSc) patients. The risk-stratification of disease severity and follow-up requires a multidisciplinary approach, integrating high-resolution computed tomography (HRTC) of the lung, pulmonary function tests (PFT), along with clinical and symptomatic evaluations. The use of HRCT in detecting SSc-ILD is not so much based on a definitive validation, but rather reflects the widespread clinician recognition of dissatisfaction with other modalities. However, due to the heterogeneity of SSc-ILD and the potential absence of symptoms in early or mild disease, it is prudent to consider as many parameters as possible in the assessment and monitoring of newly diagnosed patients. An early diagnosis meets the primary goal, i.e., the prevention of disease progression. The current first line treatment regimens are mainly centered on immunosuppressive therapy. This review assesses the role HRCT plays in optimizing care and improving clinical outcomes in SSc-ILD patients.

Keywords: high-resolution computed tomography (HRTC); interstitial lung disease (ILD); pulmonary function tests (PFT); systemic sclerosis (SSc); systemic sclerosis-associated interstitial lung disease (SSc-ILD); usual interstitial pneumonia (UIP).

Figure 1

Practical algorithm about the use of HRCT/PFT in SSc ILD.

Figure 2

Early stage. A 42-year-old female with a diagnosis of SSc. (a) Axial High Resolution CT image shows subtle ground glass opacities (black arrows) in the peripheral areas of lung bases. An additional scan with patient in prone position can be helpful to differentiate gravity-related interstitial alterations from disease-related alterations. (b) The persistence of ground-glass opacities in the prone position is related to a nonspecific interstitial pneumonia (NSIP) pattern.

Figure 2

Early stage. A 42-year-old female with a diagnosis of SSc. (a) Axial High Resolution CT image shows subtle ground glass opacities (black arrows) in the peripheral areas of lung bases. An additional scan with patient in prone position can be helpful to differentiate gravity-related interstitial alterations from disease-related alterations. (b) The persistence of ground-glass opacities in the prone position is related to a nonspecific interstitial pneumonia (NSIP) pattern.

Figure 3

Late stage. High resolution CT images in axial (a) and sagittal (b) plane show a fibrotic nonspecific interstitial pneumonia (NSIP) pattern characterized by the presence of bilateral extensive ground glass opacities (*) with a peripheral and symmetric distribution with signs of fibrosis due to the presence of reticulations (black arrows) and traction bronchiectasis (white arrows).

Figure 4

Pulmonary Hypertension. An 82-year-old female with a diagnosis of SSc. Axial High Resolution CT images show extensive subpleural reticulations (black arrows) and traction bronchiectasis/bronchiolectasis (white arrows), compatible with a UIP probable pattern (a,b). Signs of pulmonary hypertension, a common complication of this disease, can also be appreciated. On high resolution CT scan there is diffuse mosaic attenuation (*) with enlarged peripheral vessels (a,b), and a dilatation of the main pulmonary artery can be recognized using the mediastinal window setting (c).

Figure 5

Esophageal involvement in a 42-year-old female with a diagnosis of SSc. Axial image with a mediastinal window setting shows a dilatation of the esophagus (diameter >1.2 cm) with an air-fluid level.