Change of the microvascularization in systemic sclerosis, a matter of air

Abstract

Systemic sclerosis (SSc) is a rare complex disease, characterized by microvascular damage, auto-immunity, and fibrosis. Nailfold capillary microscopy (NCM), a safe and noninvasive imaging technique, can be used to visualize specific microvascular alterations in SSc. In this review, we discuss an interesting case of a patient with changes in microvascular pattern on NCM after pulmonary transplantation. We provide an overview of microvascular alterations in systemic sclerosis and the evidence in the literature about the effect of vasoactive and immunomodulation therapy on these vascular changes. We also outline the influence of pulmonal pathology, such as interstitial lung disease and pulmonary arterial hypertension, on the capillaroscopic pattern, and finally, we discuss how NCM could possibly serve as a biomarker of treatment.

Keywords: Immunomodulating therapy; Interstitial lung disease; Nailfold capillary microscopy; Pulmonary arterial hypertension; Systemic sclerosis; Vasculopathy; Vasoactive therapy.