Deregulated transforming growth factor-beta (TGF-beta) activity and responses play prominent roles in the pathogenesis of systemic sclerosis (SSc), a chronic and progressive connective tissue disease characterized by fibrosis of the skin and internal organs. Systemic sclerosis has highly heterogeneous clinical manifestations, and patients can be classified into multiple subgroups on the basis o...

Two female patients aged 62 and 44 years with progressive systemic sclerosis and passive faecal incontinence are described. Both had the typical gut motility disorders of dysphagia, heartburn, and constipation. Anorectal physiology tests showed a low resting pressure in both and an absent rectoanal inhibitory reflex in one. In both patients anal endosonography showed a thin internal anal sphinc...

results when the two ms groups were compared together, we found that cr was significantly increased (p value=0.008) and naa/cr was significantly decreased (p value=0.03) in non-enhancing lesions in ppms compared with rrms. there was no significant difference in naa, cho or naa/cho between the two ms subtypes. conclusion mrs is a potential way to differentiate ppms and rrms. objectives we compar...

Systemic sclerosis (SSc) is a complex autoimmune disorder. The cornerstones of the pathogenesis are vascular damage, fibrogenesis and various cellular and humoral autoimmune processes. The aim of the present review is to describe pathogenic steps, leading to the hallmark clinical picture of SSc. Indeed, numerous therapeutical approaches have been tested/are in use, directed towards vascular dam...

We report a case of progressive systemic sclerosis with scleroderma renal crisis 10 years after onset. The patient (female) had progressive renal dysfunction, hypertension which was difficult to control, and massive gastrointestinal bleeding. An angiotensin converting enzyme inhibitor (enalapril) could not control her hypertension. Only intravenous nicardipine had a slight effect on her hyperte...

Association between progressive systemic sclerosis (PSS) and lung malignancy is rare yet well recognised. In order of frequency bronchioloalveolar carcinoma (BAC) is most common followed by squamous cell carcinoma, oat cell carcinoma, anaplastic carcinoma of lung. We present a 55 year old non-smoker male patient with PSS, who presented to us with progressively increasing shortness of breath and...

A case is reported of progressive systemic sclerosis with pulmonary fibrosis which was complicated by recurrent haemoptyses due to diffuse pulmonary haemorrhage. We have found no other report of this association. The haemorrhage finally remitted after treatment was started with 40 mg prednisone daily, though previously spontaneous remissions had occurred.

A case of pediatric progressive systemic sclerosis is reported and a literature review concerning medical and dental aspects of this condition is provided. Systemic features include sclerodactyly, Raynaud's phenomenon, telangiectasia, calcinosis, myositis, arthritis, tenosynovitis, renal failure, esophageal hypomotility, pulmonary fibrosis and heart failure. Oral manifestations include reduced ...