Can microvascular damage predict disease severity in patients with systemic sclerosis?

Abstract

Introduction

Systemic sclerosis (SSc) is characterized by progressive fibrosis of the skin and internal organs, microvascular damage and cellular and humoral immunity abnormalities. Microvascular damage can be easily detected through nailfold videocapillaroscopy (NVC).

Materials and methods

A retrospective study of patients with SSc and a NVC performed within the first 6 months after diagnosis was conducted. Visceral involvement in the first 3 years of the disease and NVC findings were collected. The severity of microvascular damage was classified into four categories, according to the worsening of the NVC patterns. The severity of organ involvement was assessed by the disease severity scale of Medsger for each organ and as a global measure of disease severity, the simple summation was used.

Results

A total of 86 patients with SSc were included. A moderate correlation was found between the severity of microvascular damage and the global measure of disease severity (r = 0.55, p < 0.001), the severity of peripheral vascular involvement (r = 0.43, p < 0.001) and the severity of skin involvement (r = 0.34, p = 0.001).

The presence of a late scleroderma pattern in NVC were predictive in univariate analysis of digital ulcers (OR 6.03, 95% CI 1.52–23.86, p = 0.01), muscular involvement (OR 13.09, 95% CI 1.09–156.78, p = 0.04), calcinosis (OR 27.22, 95% CI 5.56–133.33, p < 0.001) and worse global disease severity score (OR 1.67, 95% CI 1.17–2.38, p = 0.005). Multivariate analysis adjusted for disease duration and gender confirmed late pattern as an independent predictor of calcinosis (OR 42.89, 95% CI 5.53–332.85, p < 0.001).

Discussion and conclusion

In this study, the worsening of NVC pattern in SSc was associated with the overall disease severity, the severity of peripheral vascular involvement and extension of skin involvement. This study highlights the importance of NVC as a prognostic tool and a possible predictor of systemic visceral involvement.