Primary pulmonary hypertension is not associated with scleroderma-like changes in nailfold capillaries

Study objectives: To determine whether primary pulmonary hypertension (PPH) is associated with scleroderma-like changes in nailfold capillaries. Design: Blinded, prospective, case-control study. Setting: University medical centers in Baltimore, MD. Patients: Thirty-seven patients with PPH, 15 patients with scleroderma, and 13 healthy control subjects. Measurements: Subjects underwent nailfold capillary videomicroscopy of the fourth digits of both hands. Capillary images were evaluated by two blinded, trained graders according to standardized criteria for the presence of scleroderma nailfold changes. Results: The prevalence of scleroderma-associated nailfold changes in patients with PPH (1 of 37 patients) was dramatically lower than that in patients with scleroderma (9 of 15 patients; p < 0.0001). The distribution of nailfold grades for the PPH patients was indistinguishable from that of the healthy control subjects. Conclusion: PPH is not associated with scleroderma-like vasculopathy of nailfold capillaries.