Progressive systemic sclerosis (SSc) is a collagen vascular disease characterised by the deposition of excessive extracellular matrix with vascular occlusion involving several organs. It commonly affects the skin (scleroderma), peripheral vasculature, kidneys, oesophagus and lungs. ILD is common in patients with SSc and causes considerable morbidity and mortality. The interstitium and pulmonary vasculature are the predominant sites that are affected. The HRCT findings of interstitial fibrosis in SSc include peripheral reticular opacities, ground-glass attenuation associated with traction bronchiectasis and occasionally honeycomb destruction (Fig. 1).
Thus, at microscopy, fibrotic NSIP is the most prevalent histological pattern in patients with SSc, whereas the UIP pattern is thought to occur in 5–10% of cases. The finding of pulmonary arterial enlargement out of proportion to the severity of the lung fibrosis may indicate an independent vasculopathy akin to primary pulmonary hypertension, and a markedly dilated oesophagus is a frequent accompaniment. Pleural disease is much less common in SSc than in other connective tissue diseases; pleural thickening is seen on HRCT in approximately 10% of patients. As in other diffuse fibrosing lung diseases, enlarged mediastinal lymph nodes (which histologically show reactive hyperplasia) are a frequent finding on CT.
Classic HRCT Findings
• Appearance compatible with fibrotic NSIP is the most common pattern of lung involvement
• Dilated oesophagus is usual
• Signs of pulmonary hypertension, often without fibrotic interstitial lung disease
FIGURE 1 ■ Scleroderma. (A) In this case, ground-glass opacification admixed with fine reticular opacities are associated with mild traction bronchiectasis. Note the mild dilatation of the oesophagus. (B) In a more advanced case, reticulation and traction bronchiectasis are more severe but still closest to an NSIP pattern; the oesophagus is also more dilated.
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