Value of Nailfold Capillaroscopy as A Screening Method for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Document Type : Original Article

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Abstract

Background: Pulmonary arterial hypertension (PAH) represents one of the main clinical pictures of microvascular damage in systemic sclerosis (SSc).
Objective: To assess the role of nailfold capillaroscopy (NFC) changes as a screening tool for the existence of PAH in patients with systemic sclerosis.
Patients and methods: Forty SSc patients were studied, included: 20 patients with PAH (diagnosed by transthoracic echocardiography and confirmed by right heart catheterization) and another 20 without PAH. History taking, clinical examination, and laboratory investigations including serological biomarkers (anti-centromere and anti-topoisomerase1 antibodies) have been recorded. NFC was performed for all patients according to the standard method.
Results: Scleroderma patients with PAH had significantly a longer disease duration (p=0.002) and higher frequencies of positive anticentromere and anti-topoisomerase 1 antibodies (p=0.011 and p=0.010 respectively). As regard NFC picture, SSc patients without PAH had significantly higher frequencies of early scleroderma pattern changes in 73.7% (p= 0.003), avascular area score 0 in 52.6% (p=0.014) and total NFC score 0 in 57.9% (p=0.022). In contrast, SSc patients with PAH showed higher frequencies of late scleroderma pattern changes in 50% (p=0.005) with lower capillary density (p <0.001), increased number of dilated mega-capillaries (p=0.049) together with more advanced avascular area of score 3 in 47.6% (p=0.007) and total NFC of score 3 in 45% (p=0.003). A significant negative correlation between mean pulmonary arterial pressure and capillary density (r=-0.57, p<0.001) was reported. Conclusion: Low capillary density together with wide avascular areas and more severe NFC scores have a clear association with the presence of PAH.

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