Bronchoalveolar lavage fluid from patients with systemic sclerosis was analysed for evidence of pulmonary vascular leakage, inflammatory cell influx, and enhanced type III collagen synthesis. Eighteen patients with systemic sclerosis and computed tomographic evidence of fibrosing alveolitis were compared with 16 patients with a normal scan. The albumin concentration in lavage fluid was higher i...

BACKGROUND Systemic sclerosis (scleroderma) is a life-threatening autoimmune disease that is characterized by the presence of specific autoantibodies and fibrosis of the skin and major internal organs. METHODS We genotyped a polymorphism (G-945C) in the promoter of the connective-tissue growth factor (CTGF) gene in 1000 subjects in two groups: group 1, consisting of 200 patients with systemic...

Single-breath carbon monoxide diffusing capacity (Dsb) was measured before and during immersion of one hand in ice water (cold pressor test) in the following three groups of subjects: (1) normal subjects; (2) patients with isolated Raynaud's disease; and (3) patients with Raynaud's phenomenon and progressive systemic sclerosis. No change in Dsb was found in normal subjects or patients with prog...

During the past 10 yrs, over 700 patients suffering from severe autoimmune disease (AD) have received an autologous haematopoietic stem cell transplant as treatment of their disorder with durable remission being obtained in around one-third. The most commonly transplanted ADs have been systemic sclerosis (scleroderma), multiple sclerosis, rheumatoid arthritis, juvenile idiopathic arthritis and ...

Objective To describe the clinical characteristics and outcomes of systemic sclerosis–mixed connective tissue disease (SSc–MCTD) SSc overlap syndrome. Methods We included patients from Australian Scleroderma Cohort Study who met American College Rheumatology/European Alliance Associations for Rheumatology criteria SSc. Three mutually exclusive groups were created: SSc–MCTD, overlap, only. Univa...

Pulmonary arterial hypertension (PAH) is an important complication of connective tissue diseases (CTD) and especially seen in systemic sclerosis, systemic lupus erythematosis (SLE), and mixed connective tissue disease (MCTD). In systemic sclerosis, PAH is isolated or accompanied by interstitial lung disease and currently, a major cause of mortality. It has been shown to be developed in approxim...

OBJECTIVE To evaluate the frequency and characteristics of ocular manifestations in outpatients with systemic sclerosis. METHODS In this cross-sectional study, 45 patients with systemic sclerosis were enrolled. Data regarding demographics, disease duration and subtype, age at diagnosis, nailfold capillaroscopic pattern and autoantibody profile were collected, and a full ophthalmic examination...

Systemic sclerosis is an autoimmune inflammatory disorder of unknown etiologycharacterized b y pronounced fibroproliferative alterations in the microvasculature, and frequent cellular and humoral immunity abnormalities, culminating in a severe and often progressive fibrotic process. Numerous biomarkers reflecting the three main pathogenetic mechanisms in systemic sclerosis have been described; ...

Skin tumors occurring on the scleroderma fingers are rarely seen. Swollen fingers are hallmarks of systemic sclerosis, and mucin deposition in the lesional skin is a constant feature in systemic sclerosis. Here we describe a case of cutaneous myxoid cyst on the flexor aspect of the sclerotic fingers in a patient with severe diffuse cutaneous systemic sclerosis. Cutaneous myxoid cyst is a relati...

Pneumatosis cystoides intestinalis (PCI) is the rare clinical finding of gas accumulation within the gastrointestinal wall. It can be secondary to a variety of disease processes including Crohn’s disease and systemic sclerosis. The present case describes PCI associated with pneumoperitoneum and ascites as the initial presentation of systemic sclerosis in a 64-year-old female with a history of C...