The rarity of scleroderma makes it a difficult disorder to study epidemiologically. Recent studies have highlighted some of the environmental exposures associated with the disease. Among occupational exposures silica remains the most important, whereas appetite suppressants might be an important drug cause. The disease also appears to cluster in space, with one report suggesting that proximity ...

Autoantibodies to components of the nucleolus are a unique serological feature of patients with scleroderma. There are autoantibodies of several specificities; one type produces a speckled pattern of nucleolar staining in immunofluorescence. In actinomycin D and 5,6-dichloro-beta-D-ribofuranosylbenzimidazole-treated Vero cells, staining was restricted to the fibrillar and not the granular regio...

Systemic sclerosis (SSc) is characterized by vascular injury, immunological abnormalities, and fibrosis of the skin as well as various internal organs. Vascular impairment is the early manifestation and plays a fundamental role in the pathogenesis of SSc. Recent studies suggest that complex interactions among the endothelial cells, pericytes, smooth muscle cells, and fibroblasts are involved in...

Scleroderma is a connective tissue disease with unknown etiology characterized by excessive deposition of extracellular matrix in the skin. Cellular infiltrates of certain immune cells and proinflammatory mediators are suggested to play a crucial role in cutaneous fibrosis, forming complicated networks between fibroblasts and immune cells via cell-cell communications. Tissue-selective trafficki...

BACKGROUND AND OBJECTIVES Scleroderma or progressive systemic sclerosis is a systemic connective tissue disease of unknown origin, which normally courses with microangiopathy, extremities ischemia and severe pain. This report aimed at describing a case of intravenous lidocaine to treat ischemic pain and at emphasizing potential anti-inflammatory action of local anesthetics in scleroderma patien...

INTRODUCTION Treprostinil diethanolamine is an innovative salt form of the prostacyclin analogue, treprostinil sodium, developed as an oral sustained release (SR) osmotic tablet. The availability of a formulation permitting convenient systemic delivery might have applicability to scleroderma vascular complications. We evaluated pharmacokinetics and perfusion in scleroderma patients with digital...

Fibrotic lung disorders, either idiopathic, or associated with a specific etiology or a specific condition such as scleroderma, are increasingly recognized. As a whole they constitute a group of diseases characterized by the progressive destruction of the lung which ultimately leads to chronic respiratory failure and death. Improving the prognosis of these disorders requires the identification ...

OBJECTIVE SPARC (secreted protein, acidic and rich in cysteine) is a matricellular protein that modulates cell-cell and cell-extracellular matrix interactions. SPARC expression is restricted mainly to sites of tissue remodelling and wound repair, and is prominent in fibrotic disorders. Single-nucleotide polymorphisms (SNPs) in the SPARC gene are reportedly linked to scleroderma in four ethnic g...

BACKGROUND Systemic sclerosis (scleroderma) is characterized by immunologic abnormalities, injury of endothelial cells, and tissue fibrosis. Abnormal oxidative stress has been documented in scleroderma and linked to fibroblast activation. Since platelet-derived growth factor (PDGF) stimulates the production of reactive oxygen species (ROS) and since IgG from patients with scleroderma reacts wit...