Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension

Approximately 20% of patients with the limited form of scleroderma will develop pulmonary hypertension which is generally a late stage fatal complication. Why pulmonary hypertension occurs in this subset of patients is unknown and it has not been possible to predict which patients are at risk. Nailfold capillary dilatation, distortion and drop occurs universally in patients with scleroderma and is generally an early finding. The present study was conducted to investigate whether quantitative nailfold capillaroscopy could distinguish those limited scleroderma patients who have established pulmonary hypertension. Quantitative nailfold capillaroscopy was performed by Visual Image Analysis in 10 healthy subjects and 20 patients with limited scleroderma (18 centromere +ve), of whom 8 had established pulmonary hypertension. It was found that scleroderma patients with pulmonary hypertension had a significant reduction in capillary density compared with patients lacking this complication (p < 0.01). Patients with scleroderma have significantly more dilated capillaries than controls although no significant differences were observe between the two patient subgroups. The finding of reduced nailfold capillary density in scleroderma patients with established pulmonary hypertension has possible pathogenic significance and may allow detection of this subgroup at an early stage in their disease progression.