Hospital Physician January 2008 33 S ystemic sclerosis, or scleroderma, is a rare connective tissue disease of unknown etiology. The word scleroderma, derived from the Greek words scleros for hard and derma for skin, refers to the hallmark finding of chronic hardening and thickening of the skin that occurs in this disease. The clinical manifestations of systemic sclerosis range from localized s...

We report a rare association of Adie's tonic pupil in a patient with systemic sclerosis who was otherwise systemically stable. This paper is an effort to unravel whether the tonic pupil and systemic sclerosis are an association by chance (which may be the case) or systemic sclerosis is the source of the tonic pupil.

14. Shulman HM, Sullivan KM, Weiden PL, et al. Chronic graft-versus-host syndrome in man. Am J Med 1980;69:204-17. 15. Kumagai Y, Shiokawa Y, Medsger TA, Rodnan GP. Clinical spectrum of connective tissue disease after cosmetic surgery. Arthritis Rheum 1984;27:1-12. 16. Maslen CL, Hall ND, Woolf AD, Maddison PJ. Enhanced oxidative metabolism of neutrophils from patients with systemic sclerosis. ...

BACKGROUND Occupational exposure to crystalline silica is a potential risk factor for various systemic autoimmune diseases including systemic sclerosis. The etiology of systemic sclerosis is not conclusively known, but there are epidemiological studies that show the relationship between exposure to crystalline silica and risk of systemic sclerosis. Here we report, for the first time, two cases ...

Our aim was to investigate the sympathetic hyperactivity of systemic sclerosis that may lead to greater morbidity and mortality from cardiovascular events. We analysed the sympathetic (low-frequency) and vagal (high-frequency) components of heart rate variability, in supine and upright positions, in 10 patients with systemic sclerosis, 12 patients with primary Raynaud's phenomenon and 14 contro...

Anti-RNA Polymerase III has been recognized as an important autoantibody in Systemic Sclerosis and it is now included in the 2013 ACR/EULAR classification criteria for Systemic Sclerosis. With this manuscript we attempt to review the current data on anti-RNA polymerase II as it relates to Systemic Sclerosis.

Pulmonary arterial hypertension is a major complication of systemic sclerosis. Although oxidative stress, intima hyperplasia and a progressive vessel occlusion appear to be clearly involved, the fine molecular mechanisms underpinning the onset and progression of systemic sclerosis-associated pulmonary arterial hypertension remain largely unknown. Here we shows for the first time that an increas...

Significant advances have been made in understanding the genetic basis of systemic sclerosis (scleroderma) in recent years. Can these discoveries lead to individualized monitoring and treatment? Besides robustly replicated genetic susceptibility loci, several genes have been recently linked to various systemic sclerosis disease manifestations. Furthermore, inclusion of genetic studies in design...

OBJECTIVE To determine the frequency and relative risk of bronchial hyperreactivity to methacholine in systemic sclerosis patients with or without associated Sjögren's syndrome. METHODS A prospective study of 56 patients with systemic sclerosis (42 with the diffuse and 14 with the limited variant; 24 with associated Sjögren's syndrome), 57 with primary Sjögren's syndrome, and 61 healthy contr...