Radiography
Posteroanterior chest radiograph reveals irregular reticular opacities in the lower lobes. Two years later, the findings are more marked.
Posteroanterior and lateral chest radiographs reveal hazy opacity in both bases, with elevation of diaphragms suggestive of restrictive lung disease.
Findings
Early findings - Subpleural reticular or reticulonodular opacity in a basal predominant distribution
Late findings
- Fibrotic changes involving the lower two thirds of the lung, with associated volume loss and honeycombing
- Pulmonary artery enlargement and cardiomegaly
Ancillary findings - Diffuse esophageal dilation with resultant "air esophagram sign" (useful when present to differentiate progressive systemic sclerosis (PSS) from idiopathic pulmonary fibrosis [IPF], asbestosis, and rheumatoid arthritis)
Degree of Confidence
Chest radiography is relatively insensitive in early disease, and findings may be minimal even in advanced disease; however, chest radiographic findings are observed in as many as two thirds of symptomatic patients, with findings suggestive of fibrosis demonstrated in 25-45% of patients.
False Positives/Negatives
Interstitial lung disease with a predominant lower lobe distribution on chest radiography may have numerous etiologies, including PSS. Clinical history and esophageal dilation on radiographs suggests PSS. Any phase of IPF, asbestosis, interstitial pulmonary edema, lymphangitic metastases, pulmonary hemorrhage, and other diffuse lung diseases may mimic PSS on chest radiographs. On the posteroanterior (PA) view, the breast shadows can mimic increased markings over the lower lung zones. Lateral view is critical so as to obtain a less obscured view of the lower lobes.
Computed Tomography
High-resolution CT of biopsy-proven nonspecific interstitial pneumonia. The image shows abnormal reticular opacities in a background of ground-glass opacity, with associated traction bronchiectasis indicating lung fibrosis.
High-resolution CT demonstrates extensive basal honeycombing suggestive of lung fibrosis. Note the extraluminal peritoneal cavity air presumed to be secondary to benign pneumatosis intestinalis and the dilated esophagus with an air-fluid level.
Findings
Patients with suggested or known diagnosis of PSS should undergo the following13 :
Standard resolution CT (SRCT) to exclude a lung neoplasm (which is slightly more common than in the general population) and to evaluate pleuropericardial, esophageal, cardiac, and pulmonary arterial abnormalities
HRCT to evaluate lung parenchymal involvement
- Imaging findings
- Pulmonary fibrosis and pulmonary hypertension are the most common findings.
- Overlap syndromes or CREST syndrome are suggested when pulmonary hypertension is out of proportion to the degree of fibrosis.
- HRCT findings of pulmonary fibrosis include the following:
- Architectural distortion
- Subpleural cysts or honeycombing
- Irregular reticular opacities
- Interlobular septal thickening
- Traction bronchiectasis and bronchiolectasis
- Visceral pleural thickening
- Parenchymal micronodules
- Ground-glass opacity may indicate acute inflammation (alveolitis).
- Honeycombing favors a histologic usual interstitial pneumonitis (UIP) pattern.
- Mixed reticulation and ground-glass opacity with little or absent honeycombing favor histologic nonspecific interstitial pneumonia (NSIP).
- Distribution of disease
- Fibrosis is subpleural and basal predominant.
- A less marked involvement of the upper lobes favors PSS or other collagen vascular disease as the etiologic cause of the UIP pattern rather than IPF or chronic hypersensitivity pneumonitis.
- Ancillary findings
- Mediastinal adenopathy is found, with increased prevalence in more extensive lung disease.
- Esophageal dilation is present on CT in 80% of patients with diffuse lung disease resulting from PSS.
Degree of Confidence
Studies have shown that high-resolution CT (HRCT) is diagnostically accurate in specific subsets of patients with diffuse lung disease. In a study of 85 patients with diffuse lung disease by Swensen et al,14 radiologists more frequently had a high level of confidence in diagnosing UIP than any other pattern and listed it as the number one diagnosis in 89% of patients with that histologic finding. Imaging diagnoses were most accurate (90% correct) when a high level of confidence was present.
Remy-Jardin and colleagues correlated HRCT findings with functional parameters and bronchoalveolar lavage (BAL) results in patients with PSS.12 BAL findings were abnormal in 7 of 21 patients with normal imaging and PFTs, suggesting it is more sensitive than HRCT in detecting subclinical alveolitis. The extent of parenchymal destruction, which correlated with functional abnormality, was better depicted by HRCT.
Diot and colleagues assigned point values for each abnormal finding present on HRCT of 52 patients with progressive systemic sclerosis (PSS).15 Total scores were inversely proportional to total lung capacity and diffusing capacity for carbon monoxide (DLCO). Receiver operator curve analysis demonstrated that an HRCT score of 7 or greater minimized false negatives (sensitivity of 0.60) while achieving a specificity of 0.83. The positive predictive value of HRCT in this study was 0.82.
False Positives/Negatives
False negatives have been documented by Remy-Jardin et al in patients with subclinical alveolitis.12
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Further Reading
Related eMedicine topics:
Scleroderma (from Rheumatology)
Systemic Sclerosis
Localized Fibrosing Disorders: Linear Scleroderma, Morphea, Regional Fibrosis
CREST Syndrome
Mixed Connective-Tissue Disease
Clinical Trial:
Effectiveness and Safety of Lidocaine for Scleroderma
Keywords
thoracic scleroderma, progressive systemic sclerosis, PSS, scleroderma, SSc, diffuse cutaneous systemic sclerosis, limited cutaneous systemic sclerosis, dcSSc, lcSSc
