The aim of this study was to determine the prevalence of enlarged mediastinal lymph nodes in systemic sclerosis (SSc) and relate this to the extent of pulmonary disease and disease activity as judged by high resolution computed tomography (HRCT). The HRCT scans of 78 patients with SSc were reviewed. The extent of lung disease and HRCT pattern were analysed and CT scans examined on soft tissue window settings for evidence of mediastinal lymph node enlargement. Sixty six (85%) patients had evidence of lung involvement on CT. Enlarged mediastinal lymph nodes were present in 21 (32%) patients with lung involvement but in only 1 (8%) patient without. The prevalence of enlarged mediastinal nodes increased with more extensive lung involvement on CT (p < 0.025), but correlated poorly with the type of CT appearance and concurrent erythrocyte sedimentation rate. Mediastinal lymph node enlargement occurs frequently in patients with SSc, particularly if lung involvement is extensive.