Vascular Manifestations of Systemic Sclerosis

Abstract

　Various manifestations are seen in the skin and internal organs of systemic sclerosis patients. The pathogeneses of these manifestations are considered to involve fibrosis, autoimmune dysfunction, and/or vasculopathy.

　Almost all patients have skin symptoms, which are classified into two groups: fibrotic lesions and vascular lesions. The fibrotic lesions include skin fibrosis, short sublingual frenulum, microstomia, mask-like face, and contracture of the phalanges, while Raynaud's phenomenon, skin ulcers, nail fold bleeding, pitting scars, telangiectasias, and pulmonary hypertension are well-known vascular lesions. These symptoms are present throughout the course of the disease and can severely affect patients' quality of life. However, their causative mechanisms are still to be clarified.

　Thus, this review aims to describe our current understanding of the clinical features of the vascular manifestations of systemic sclerosis, and discusses their pathogenic mechanisms and treatment.