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...

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...

In patients with progressive systemic sclerosis, impaired motor function of the small intestine may lead to bacterial overgrowth causing diarrhoea, steatorrhoea and malabsorption. As unconjugated serum bile acids have been proposed as markers for small bowel bacterial overgrowth, we studied individual unconjugated serum bile acids in 36 patients with progressive systemic sclerosis. These patien...

BACKGROUND Systemic sclerosis (SSc), a progressive disease characterized by excessive accumulation of connective tissue components. Although most patients have long survival, some of them progress rapidly to death. Pulmonary system involvement and pulmonary hypertension are the most frequent cause of death. When the patient with SSc is to be operated, the anesthetic procedure could be a serious...

Background: Systemic sclerosis ( SSc ), a progressive disease characterized by excessive accumulation of connective tissue components. Although most patients have long survival, some of them progress rapidly to death. Pulmonary system involvement and pulmonary hypertension are the most frequent cause of death. When the patient with SSc is to be operated, the anesthetic procedure could be a seri...

In this report, we describe the use of extracorporeal photochemotherapy in n the treatment of two patients with rapidly advancing progressive systemic sclerosis. Both patients experienced improvement in the cutaneous as well as the systemic manifestations of their disease while undergoing therapy. The potential therapeutic mechanisms are discussed.

A 31-year-old woman with a prior history of an overlap syndrome of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) developed fever, pericarditis with pericardial effusion and a rapidly progressive fatal interstitial lung disease. Diagnostic test and procedures, differential diagnosis and therapeutic approach are discussed.

In recent years, many potential antifibrotic treatment strategies have emerged from molecular studies of systemic sclerosis. Few biologicals have already entered clinical trials and these may hopefully prove to be effective in this progressive, profibrotic disease.

Morphoea is a chronic inflammatory disease of the skin and underlying tissues, characterised by fibrosis subcutaneous tissue without systemic involvement. Radiation-induced morphoea rare, often unrecognised, chronically progressive form radiation-associated localised scleroderma skin, infrequently progressing to scleroderma. Systemic widespread along with features, leading an increased risk mal...