Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sc...

BACKGROUND It would be a benefit if time-saving, non-invasive methods could give hints for diagnosing systemic sclerosis. To investigate the skin of patients with systemic sclerosis using confocal laser scanning microscopy in vivo and to develop histometric parameters to describe characteristic cutaneous changes of systemic sclerosis observed by this new technique, we conducted an exploratory s...

Multiple pulmonary malignancy is uncommon and has not been reported in association with systemic sclerosis. We now report such a case in which it is likely that the connective tissue disease occurred as a marker of the internal malignancy. Systemic sclerosis is not generally a reliable indicator of malignant change, but it is possible that in patients who are immunosuppressed, either therapeuti...

Chronic intestinal pseudo-obstruction is a known complication of patients with systemic sclerosis, manifested as nausea, vomiting, constipation, abdominal distension and pain. We report a series of cases with systemic sclerosis that presented with signs of intestinal obstruction. In all cases, the differentiation between a pseudo-obstruction and true mechanical obstruction remained a formidable...

Three cases of systemic sclerosis in which trigeminal neuropathy was the presenting symptom are described. All 3 patients had progressive diseases and 2 died from its complications. In each case extensive neurological investigation was undertaken before the disease was recognized and it is suggested that earlier recognition of the systemic sclerosis might obviate the need for this. Trigeminal n...

Abstract: Systemic sclerosis is a rare connective tissue disease categorized by extensive lung fibrosis, vascular and immunologic abnormalities. Autoimmune antibodies like anticentromere, anti-Scl-70 (anti-topoisomerase I), anti-RNA polymerase II are commonly involved in progressive disease. Clinical presentations consist of Raynaud’s phenomenon, digital ulcers; pericardial effusion, telangiect...

The aim of this study was to assess left ventricular function in subjects with systemic sclerosis. Twenty-four women with systemic sclerosis (mean age 48 ___ 11 yr) and 14 ageand sex-matched normal subjects were studied by radionuclide angiography performed at rest with a temporal resolution of 20 msec/frame. Left ventricular volume curves were generated and indices of systolic and diastolic fu...

BACKGROUND Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vascular alterations and autoimmune activation leading to widespread organ fibrosis. At the early stage of disease when organ involvement and extent of disease are emerging, mast cells may have some role, as implied by both symptoms and histologic evidence. CASE PRESENTATION A female patient diagnosed with cu...

The aim of this study was to assess left ventricular function in subjects with systemic sclerosis. Twenty-four women with systemic sclerosis (mean age 48 +/- 11 yr) and 14 age- and sex-matched normal subjects were studied by radionuclide angiography performed at rest with a temporal resolution of 20 msec/frame. Left ventricular volume curves were generated and indices of systolic and diastolic ...