Abstract

Peripheral microvascular impairment in systemic sclerosis (SSc) may be easily detected and scored in a safe noninvasive way by nailfold videocapillaroscopy (NVC). The paper highlights clinical conditions related to SSc in which NVC may represent an outcome measure of therapeutical interventions, by elaborating on their already assessed relationship with the NVC patterns and eventually scores. The 3 important biological/clinical conditions are: the positivity for SSc-specific serum autoantibodies, the presence of SSc skin digital ulcers (DUs) and of pulmonary arterial hypertension (PAH) SSc associated. In conclusion, to the question if capillaroscopy (NVC) may represent in SSc an outcome measure for clinical trials on the peripheral vasculopathy, based on the growing evidence and our detailed studies, the answer is positive. Recent therapeutic trials in SSc are confirming this role, and the experience is growing rapidly.

Highlights

  • Systemic sclerosis (SSc) is characterized by early and persistent microvascular impairment leading to functional Raynaud’s phenomenon (RP) and clinical manifestations (Figure 1) [1, 2].Digital ulcers in systemic sclerosis (SSc) are considered to be related to tissue ischemia following several processes, including at the beginning persistent vasospasm (RP), but in the progression of the disease to intimal fibroproliferation, tissue fibrosis, and thrombosis of digital arteries [3].Progressive deficiency in vasodilatory capacity of the vessels and tissue fibrosis is proposed as a mechanism of the persistent vascular spasm; the mechanism of endothelial injury is still unclear [4].The assessment of vascular involvement is still a matter of study, and several noninvasive techniques have been proposed

  • The paper highlights clinical conditions related to SSc in which nailfold videocapillaroscopy (NVC) may represent an outcome measure of therapeutical interventions, by elaborating on their already assessed relationship with the NVC patterns and eventually scores

  • Digital ulcers in SSc are considered to be related to tissue ischemia following several processes, including at the beginning persistent vasospasm (RP), but in the progression of the disease to intimal fibroproliferation, tissue fibrosis, and thrombosis of digital arteries [3]

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Summary

Introduction

Systemic sclerosis (SSc) is characterized by early and persistent microvascular impairment leading to functional Raynaud’s phenomenon (RP) and clinical manifestations (i.e., digital ulcers, pulmonary arterial hypertension, etc.) (Figure 1) [1, 2]. The morphological capillary abnormalities in SSc have been classified in 3 validated patterns (early, active, and late) of microangiopathy by NVC and scored (Figure 2) [5,6,7]. Laser Doppler flowmetry (LDF) is the best non invasive and safe technique to assess and to measure the blood perfusion at peripheral sites [8, 9]. Patients with SSc showing the late NVC pattern of microangiopathy have a significantly lower finger blood perfusion (FBP) than patients with the active and early NVC patterns (P < .05) [10]. The 3 important biological/clinical conditions are: the SSc-specific serum autoantibodies, the SSc skin digital ulcers (DUs), and the pulmonary arterial hypertension (PAH) associated to SSc

Serum Autoantibodies and NVC
Skin Digital Ulcers and NVC
Findings
Pulmonary Arterial Hypertension and NVC
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