PURPOSE OF REVIEW Animal models have been extremely valuable in contributing to a better understanding of the pathogenesis of systemic sclerosis. Discussed here are recent studies that have examined the molecular pathways and potential therapeutic approaches for systemic sclerosis using animal models. RECENT FINDINGS Reported evidence further indicates that the immune system plays a role in m...

Two aspects of generalised scleroderma have received much attention in recent years. Firstly, the widespread and sclerotic nature of the disease process has been embodied in the term “progressive systemic sclerosis,” proposed by Goetz’ in 1945, and since adopted by other authors. Secondly, connective tissue alterations are now regarded as an integral part of this entity which answers all the cr...

BACKGROUND Increased expression of CD40L has been reported on activated CD4+ T lymphocytes in systemic sclerosis. CD40L can be expressed in soluble form (sCD40L). OBJECTIVE To compare sCD40L concentrations in patients with systemic sclerosis and healthy controls. METHODS Quantitative sandwich ELISA was used to measure plasma sCD40L in systemic sclerosis (n = 50) and matched healthy controls...

The case is described of a patient with scleromyxoedema with features typical of systemic sclerosis. The features were so characteristic that the disease was misdiagnosed as systemic sclerosis. A brief review of the association of the two diseases is given.

The activation threshold of B cells is tightly regulated by an array of inhibitory and activator receptors in such a way that disturbances in their expression can lead to the appearance of autoimmunity. The aim of this study was to evaluate the expression of activating and inhibitory molecules involved in the modulation of B cell functions in transitional, naive, and memory B-cell subpopulation...

OBJECTIVE Salvia miltiorrhiza has long been used to treat systemic sclerosis. Tanshinone IIA, one of the phytochemicals derived from the roots of Salvia miltiorrhiza, exhibits multiple biological activities. The present study aimed to investigate whether tanshinone IIA has an effect on the interleukin-17A-induced functional activation of systemic sclerosis patient-derived dermal vascular smooth...

The safety of chemotherapy for patients with systemic sclerosis is unclear, and there are few published reports documenting the side effects of chemotherapy in patients with this condition. Here, we report the case of a patient with systemic sclerosis who developed severe digital ischemia during combination gemcitabine/S-1 chemotherapy for pancreatic cancer. In spite of aggressive treatment, th...

Background: Systemic sclerosis is an autoimmune disease affecting connective tissues, (including epidermal, subepidermal, microvasculature, etc.), leading to various extent of end-organ damage. The leading cause of mortality among these patients is lung involvement. The cardiovascular events happen more frequently in patients suffering systemic scleroderma, comparing to healthy population. This...

OBJECTIVES To investigate the behaviour of circulating endothelin-1 (ET-1) in patients affected by systemic sclerosis and to elucidate the relationship between systemic and pulmonary plasma peptide and arterial pressure levels. METHODS Plasma ET-1 concentrations were determined in 48 patients affected by systemic sclerosis (41 women, seven men; mean age 47.2 (SD 5.5) years) with or without sy...