OBJECTIVES The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). METHODS The DUO Registry is a European, prospective, multicentre, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regi...

BACKGROUND Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004....

  Primary Hepatic Lymphoma (PHL) is rare and possibly associated with viral hepatitis and autoimmune diseases. Scleroderma could exceptionally be complicated by lymphoma. We describe PHL occurring in a 52-year-old female suffering scleroderma for eight years, with no history of cytotoxic or high-dose glucocorticoid therapy. CT scan, performed to work-up abdominal discomfort, constipation, and ...

primary hepatic lymphoma (phl) is rare and possibly associated with viral hepatitis and autoimmune diseases. scleroderma could exceptionally be complicated by lymphoma. we describe phl occurring in a 52-year-old female suffering scleroderma for eight years, with no history of cytotoxic or high-dose glucocorticoid therapy. ct scan, performed to work-up abdominal discomfort, constipation, and ele...

The aim of the present study was to investigate the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma-associated pulmonary arterial hypertension (PAH) and healthy controls. PAH was documented by right-heart catheterisation (mean pulmonary artery pressure at rest >25 mmHg). Serum immunoglobulin (Ig)G and IgM reactivities of patients with idiopathic PAH (n = 35), s...

INTRODUCTION In systemic sclerosis (SSc) little evidence for the effectiveness of anti-inflammatory and immunosuppressive therapy exists. The objective of this study was to determine the extent to which SSc patients are treated with corticosteroids and immunosuppressive agents. METHODS Data on duration and dosage of corticosteroids and on the type of immunosuppressive agent were analyzed from...

Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tiss...

OBJECTIVE To determine the natural history and timing of severe involvement of the kidney, heart, lung, gastrointestinal (GI) tract, and skin in patients with systemic sclerosis (SSc) and diffuse cutaneous involvement. METHODS This study used the Pittsburgh Scleroderma Databank and included patients with diffuse scleroderma who were seen between January 1, 1972 and December 31, 1995. Patients...

SSc can be associated with a high morbidity and mortality. Due to the complexity and heterogeneity of the disease, a composite response measure that will capture differing organ involvements and patient-reported outcomes is desirable. Recently, with participation of the Scleroderma Clinical Trial Consortium, a standardized core set of items were proposed for SSc clinical trials using a structur...

More than 300 sera from patients with a connective tissue disease were analyzed with the immunoblotting technique. The presence of autoantibodies against an 86,000-mol wt marker antigen for diffuse scleroderma (Scl-86) was found in 14 out of 33 patients with scleroderma. The presence of anti-Scl-86 antibodies seemed to correlate with the diagnosis of diffuse scleroderma since they were found in...