Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
June 16th, 2020
Here, we present practical recommendations for performing thoracic high-resolution computed tomography for diagnosing and assessing systemic sclerosis-related interstitial lung disease.
This HRCT protocol is important for diagnosing systemic sclerosis related interstitial lung disease. And can be used for one off scanning or sequential scanning over time. It can help guide treatment decisions.
HRCT is considered the most sensitive non-invasive means for facilitating the diagnosis of systemic sclerosis related interstitial lung disease, and can be used to protect the future course of lung disease and therapeutical response. To confirm the target area, first set the HRCT scanner parameters as outlined in the table, while giving breathing instructions to the patient. Take a deep breath in and blow it out.
Take another deep breath in, and blow it out. Acquire a volumetric exploratory metric scan from the lung apices to the lung base. Next, acquire a sequential supine exploratory scan with 10 to 20 millimeter gaps from two centimeters below the lung apices to the lung base, followed by a sequential prone inspiratory optional with 10 to 20 millimeter gaps from the carina for the lung base.
For optimal quality volumetric scans, obtain less than two millimeter images with a high spatial resolution reconstruction. Review each scan image immediately after acquisition. And repeat the scan if motion artifacts are present, or if an inadequate inspiration has occurred.
When all of the scans have been acquired, prepare an interpretive report, and share the report and images with the patient's care team and medical records. Key features of SSc-ILD as assessed by HRCT commonly include a nonspecific interstitial pneumonia pattern with peripheral ground glass opacities and extensive traction bronchiectasis. As observed in these images, central predominance or peripheral distribution with subpleural sparing is highly suggestive of nonspecific interstitial pneumonia.
Typically, traction bronchiectasis and traction bronchiectasis are often the predominant features of systemic sclerosis related interstitial lung disease. Additional findings may include honeycombing, which is observed more commonly in diffuse forms of systemic sclerosis, and a dilated air-filled esophagus. A pulmonary artery size greater than the adjacent ascending aorta suggests a coexistence, pulmonary hypertension, even in patients without fibrotic lung disease.
Here are two sets of axial and coronal chest HRCT images taken 10 years apart in a patient with systemic sclerosis associated interstitial lung disease can be observed. The initial axial and coronal images demonstrate a basilar predominant ground glass opacity and reticulation with mild traction bronchiectasis and subpleural sparing consistent with nonspecific interstitial pneumonia. In the images obtained 10 years later, an increased reticulation and traction bronchiectasis at the lung bases with a decrease in the ground glass opacity that is consistent with a mild worsening of pulmonary fibrosis, can be observed.
Be sure to acquire supine inspiration, crone inspiration, and expiration images without motion artifact using thin reconstruction or acquisition slice thickness. Asking patients to take two or three breaths can be beneficial, forced vital capacity assessments, can reform in correlation with HRCT to assess a patient's disease stage per the ghost agent system. And it can be helpful in making treatment decisions.
Overview
This article presents practical recommendations for performing thoracic high-resolution computed tomography (HRCT) to diagnose and assess systemic sclerosis-related interstitial lung disease (SSc-ILD). The HRCT protocol is crucial for guiding treatment decisions and monitoring disease progression.
Key Study Components
Area of Science
- Neuroscience
- Radiology
- Pulmonology
Background
- Systemic sclerosis can lead to interstitial lung disease.
- HRCT is the most sensitive non-invasive method for diagnosis.
- Understanding HRCT findings is essential for treatment planning.
- Key features include ground glass opacities and traction bronchiectasis.
Purpose of Study
- To provide a standardized HRCT protocol for SSc-ILD diagnosis.
- To enhance the accuracy of treatment decisions based on imaging.
- To facilitate monitoring of disease progression over time.
Methods Used
- Set HRCT scanner parameters as outlined.
- Instruct patients on breathing techniques for optimal imaging.
- Acquire volumetric scans from lung apices to bases.
- Review and interpret scans immediately after acquisition.
Main Results
- Initial scans show ground glass opacities and traction bronchiectasis.
- Follow-up scans indicate changes consistent with disease progression.
- HRCT findings correlate with clinical assessments of lung function.
- Key imaging features aid in distinguishing disease types.
Conclusions
- HRCT is vital for diagnosing and managing SSc-ILD.
- Regular imaging can track disease progression effectively.
- Standardized protocols improve diagnostic accuracy and treatment outcomes.
Frequently Asked Questions
Executive Industry Relevance
High-resolution computed tomography (HRCT) provides critical imaging biomarkers for systemic sclerosis-associated interstitial lung disease (SSc-ILD), enabling early detection and longitudinal monitoring of fibrotic progression. This supports target validation in preclinical models by establishing disease-relevant imaging endpoints that correlate with pulmonary function and mortality risk. Standardized HRCT protocols enhance predictive confidence in therapeutic screening by quantifying treatment effects on lung parenchyma across discovery and translational stages.
Strategic Applications in Biopharma R&D
Early Discovery & Target Validation
- Scientific Value: Enables interrogation of therapeutic hypotheses by visualizing NSIP pattern, ground-glass opacities, and traction bronchiectasis as disease-specific imaging phenotypes.
- Operational Value: Supports biological de-risking through objective, quantitative assessment of fibrotic burden and progression in disease models.
- Predictive Value: Facilitates portfolio triage by identifying models with high fibrosis score or extensive disease (>20%) linked to increased mortality risk.
Screening & Assay Development
- Assay Readiness: Prepares validated imaging readouts for downstream compound evaluation by establishing baseline and serial HRCT metrics.
- Quantitative Outputs: Enables measurement of ground-glass opacity proportion, fibrosis coarseness, and esophageal dilation (>10 mm) as translatable biomarkers.
- Platform Reuse: Supports scalable, reproducible imaging workflows applicable across preclinical and clinical candidate evaluation.
Translational & Preclinical Research
- Disease Relevance: Distinguishes SSc-ILD from idiopathic pulmonary fibrosis via greater ground-glass opacity and less coarse fibrosis, supporting mechanism-specific target validation.
- Translational Continuity: Connects discovery findings to preclinical validation through serial HRCT monitoring of treatment response and disease progression.
- Risk-Adjusted Decisions: Informs advancement criteria by correlating pulmonary artery enlargement (>ascending aorta) with pulmonary hypertension risk and nodule monitoring with lung cancer surveillance.
Pipeline & Workflow Integration
HRCT imaging functions as a translational bridge from early discovery through preclinical validation, providing quantitative, disease-relevant endpoints that support go/no-go decisions in fibrosis-focused drug development programs.
- Discovery Biology: Supports hypothesis testing by visualizing NSIP pattern and traction bronchiectasis as mechanistic readouts of fibrotic pathways.
- Screening: Delivers assay-ready, reproducible imaging data with standardized acquisition and reconstruction protocols for compound screening campaigns.
- Analytics: Provides quantitative dependent variables including fibrosis score, ground-glass opacity percentage, and vascular ratios for statistical comparison across treatment groups.
- Translational Research: Ensures continuity from target engagement to phenotypic outcome via longitudinal imaging aligned with clinical assessment systems.
- Enterprise Reuse: Establishes a standardized, cross-functional imaging capability applicable to multiple fibrotic and autoimmune disease programs.
Operational & Enterprise Impact
- Scientific Value: Increases predictive confidence by reducing mechanistic ambiguity through direct visualization of disease-specific fibrotic patterns.
- Operational Value: Enhances standardization and reproducibility via defined scanning parameters, breath-hold techniques, and immediate quality review.
- Strategic Value: Improves capital efficiency by enabling early go/no-go decisions based on imaging biomarkers of progression and mortality risk.
- Portfolio Impact: Supports risk-adjusted prioritization through objective thresholds such as fibrosis score and disease extent (>20%) linked to clinical outcomes.
Implementation Considerations
- Requires expertise in thoracic imaging acquisition and interpretation for accurate NSIP pattern recognition and fibrosis quantification.
- Depends on high-resolution CT scanners capable of sub-millimeter slice thickness and high-spatial-frequency reconstruction.
- Necessitates cross-team standardization of breathing protocols and scan timing to minimize motion artifacts and ensure inspiratory consistency.
- Involves adaptation considerations when translating HRCT protocols across species or disease models with varying respiratory mechanics.
- Includes practical limitations such as ionizing radiation exposure, which may be mitigated through low-dose techniques and justified by diagnostic yield.
