Raynaud’s phenomenon (RP) is one of the early clinical manifestations of diffuse connective tissue diseases (DCTD), such as systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), and dermatomyositis (DM)/ polymyositis (PM), and nailfold capillaroscopy (NC) enables an in vivo assessment of the morphology of cutaneous capillaries, and has been used f...

OBJECTIVE To examine the predictive role of HLA genetic markers in scleroderma renal crisis (SRC), beyond the known clinical correlates, in a large population of patients with systemic sclerosis (SSc). METHODS SSc patients from the Scleroderma Family Registry and DNA Repository, the Genetics versus Environment in Scleroderma Outcomes Study, and the rheumatology division registry at the Univer...

Cell-mediated immunity to skin extracts was studied by the macrophage migration inhibition test, lymphocyte transformation, and direct cytotoxicity to skin fibroblasts, in normal individuals and patients with progressive systemic sclerosis. The latter included 18 individuals with diffuse scleroderma and 12 with the CREST syndrome, a variant form of systemic sclerosis in which there is more limi...

INTRODUCTION Gastrointestinal (GI) involvement is a serious complication of systemic sclerosis (SSc). OBJECTIVES The aim of the study was to determine the incidence of GI manifestations in SSc. PATIENTS AND METHODS We studied 73 patients with SSc (60 women and 13 men). Diffuse cutaneous SSc (dcSSc) was diagnosed in 30 patients and limited cutaneous SSc (lcSSc) in 43 patients. Upper GI invol...

OBJECTIVES To identify clinical, functional and health-related quality of life (HRQoL) correlates of clinically significant symptoms of anxiety and depression in patients with systemic sclerosis (SSc). METHODS Three-hundred-and-eighty-one patients fulfilling the American College of Rheumatology and/or the Leroy and Medsger criteria for SSc were assessed for visceral involvement, disability an...

The association of exposure to bleomycin with the development of scleroderma-like cutaneous abnormalities has been reported. We experienced a case of scleroderma involving the hands, feet, and forearms after bleomycin chemotherapy. The present report supports the possible causal relation of bleomycin with scleroderma. Regarding the widespread use of bleomycin, this complication is thought to be...

S ystemic sclerosis (SSc) is a systemic autoimmune disease that is characterised by endothelial dysfunction resulting in a small-vessel vasculopathy, fibroblast dysfunction with resultant excessive collagen production and fibrosis, and immunological abnormalities. The classification of SSc is subdivided based on the extent of skin involvement into diffuse cutaneous sclerosis (dcSSc), limited cu...

BACKGROUND Previous studies have suggested an increased risk of cancer among patients with scleroderma. OBJECTIVE To study a population based cohort of patients with scleroderma in South Australia. METHODS Subjects with scleroderma were identified from the South Australian Scleroderma Registry established in 1993. All subjects on the scleroderma registry were linked to the South Australian ...

A 47‐year‐old woman with mixed connective tissue disease was treated abatacept. After 2 months, a 3‐cm depression atrophied surface observed on her back, which had histopathological consistent the symptoms of localized scleroderma. Although some cases paradoxical reaction or cutaneous adverse event have been reported from abatacept, no scleroderma has reported, suggesting this to be unique case.

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...