BACKGROUND Atrial conduction abnormalities in patients with scleroderma have not been evaluated in terms of P wave duration, P wave dispersion (P(d)) and electromechanical coupling measured by tissue Doppler echocardiography. METHODS Twenty-four patients with scleroderma and 24 control subjects underwent resting electrocardiogram (ECG), M mode and tissue Doppler echocardiography. The P wave d...

Transient exposure to inflammation-associated, fibroblast-stimulatory factors appears to initiate fibrosis by inducing the persistently activated phenotypes displayed by fibroblast cultures derived from scleroderma skin and other fibrotic tissues. To determine whether one class of fibroblast-inhibitory factors, the interferons (IFNs), plays a role in terminating fibrosis by acting as persistent...

Autoimmune diseases are thought to be initiated by exposures to foreign antigens that cross-react with endogenous molecules. Scleroderma is an autoimmune connective tissue disease in which patients make antibodies to a limited group of autoantigens, including RPC1, encoded by the POLR3A gene. As patients with scleroderma and antibodies against RPC1 are at increased risk for cancer, we hypothesi...

Sir, Linear scleroderma represents a rare disease, more often observed in the pediatric age group. The lower extremities are usually involved; less commonly it affects the frontoparietal area and the anterior scalp. Recently, a large case series of frontoparietal scleroderma en coup de sabre has been reported (1), but in this series, as well as in the English language literature, to the best of...

Scleroderma is a fibrotic condition characterized by immunological abnormalities, vascular injury and increased accumulation of extracellular matrix proteins in the skin. Although the etiology of scleroderma has not been fully elucidated, a growing body of evidence suggests that the overproduction of extracellular matrix proteins by activated fibroblasts results from an imbalance between synthe...

A 67-year-old woman with a 15-year history of intestinal scleroderma presented with recurrent melena. Upper endoscopies revealed a healing, non-bleeding, large gastric ulcer. After the third bleed, angiography demonstrated bleeding from a splenic artery pseudoaneurysm adjacent to the gastric ulcer. Scleroderma patients are at risk of bleeding from esophagitis or gastric arteriovenous malformati...

Prof. Alan Tyndall, Secretary, World Scleroderma Foundation, Basel, Switzerland; Ann Tyrrell Kennedy, President, FESCA, Blackrock, Co. Dublin, Ireland; Prof. Dinesh Khanna, Director, University of Michigan Scleroderma Program, Ann Arbor, MI, USA; Prof. Yannick Allanore, President, EUSTAR, Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France. Please address corre...

A postal questionnaire was used in a nationwide case control study of women with scleroderma to investigate whether there was an increased rate of spontaneous abortion before the onset of the disease. The results from 155 case control pairs studied showed that the women with scleroderma had twice the rate of spontaneous abortion and three times the rate of fertility problems (no successful preg...

Department of Clinical Immunology and Rheumatology, Sahara Hospital, Lucknow, India, 226010 [email protected] Scleroderma or progressive systemic sclerosis is diagnosed clinically by typical features of skin thickening, Raynaud’s phenomenon and visceral organ involvement, and serologically by distinct autoantibody subsets. These differentiate the disease into the ‘limited’ and ‘diffuse’ vari...

Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic r...