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.