Dysexecutive Syndrome A Specific Pattern of Cognitive Impairment in Systemic Sclerosis PDF Print E-mail
Sunday, 07 July 2013 20:31
Systemic sclerosis (SSc), also called scleroderma, is a connective-tissue disorder characterized by obliterative microvascular lesions and diffuse interstitial fibrosis. SSc damages the small and medium-sized vessels serving the skin, joints (especially fingers and toes), and internal organs such as the lungs, gastrointestinal tract, kidneys, and heart. In most patients, neurological involvement is limited, and localized to the peripheral nervous system. Raynaud phenomenon (RP) is a prominent early vascular feature of SSc, affecting 90% of patients during their disease course and, in some, progressing
to severe ischemia.
 
Frequency of Sexual Dysfunction In Women With Rheumatic Diseases PDF Print E-mail
Sunday, 07 July 2013 20:08
Sexuality is part of human life and of quality of life, accounting for individual well-being. It relates not only to sexual intercourse itself, but also to a whole spectrum that ranges from self-image and self-valuing to relationship with the ‘Other’. Appropriate sexual activity comprises phases from sexual arousal to relaxation, with pleasure and satisfaction.

Sexual dysfunction is a change in a phase of the sexual activity that can culminate in frustration, pain, and a reduction in the number of sexual intercourses. Some studies have shown a prevalence of sexual dysfunction in the general female population of as much as 40%. Chronic diseases are known to influence the quality of sexual life, but their effect is little studied, and sexual dysfunction, little diagnosed.
 
Interleukin-6: A New Therapeutic Target in Scleroderma PDF Print E-mail
Sunday, 12 May 2013 19:15
Interleukin-6 (IL-6) is a classic pro-inflammatory cytokine critical in mounting an effective immune response. It is secreted by a wide array of cell types; however, its effector cells are more restricted, owing to the fact that very few cells, except lymphocytes and hepatocytes, express the functional membrane IL-6 receptor thus reducing the number of IL-6-responsive cells. Transsignalling, the shedding of the membrane-bound form of the IL-6 receptor into the local microenvironment, greatly increases the range of cells that can respond. IL-6 has been demonstrated to have a pivotal role in the pathogenesis of rheumatoid arthritis, Castleman’s disease and Crohn’s disease exemplified by the use of an anti-IL-6 biological therapy.
 
An Approach to the Treatment of Scleroderma PDF Print E-mail
Wednesday, 03 April 2013 22:30
Systemic sclerosis is unique among the rheumatic diseases because it presents the challenge of managing a chronic multisystem autoimmune disease with a widespread obliterative vasculopathy of small arteries that is associated with varying degrees of tissue fibrosis.

The hallmark of Scleroderma is clinical heterogeneity with subsets that vary in the degree of disease expression, organ involvement, and ultimate prognosis. Thus, the term Scleroderma is used to describe patients who have common
 
Systemic Sclerosis: An Update PDF Print E-mail
Friday, 08 March 2013 00:33
Systemic sclerosis (SSc) is a clinically heterogeneous, systemic disorder affecting connective tissue of skin, internal organs, and walls of blood vessels. It is characterized by alterations of the microvasculature in the form of hypoxia, digital ulcers, and pulmonary arterial hypertension; disturbances of the immune system, including dysbalance of cytokine expression, autoantibodies (Auto-ab), and abnormalities of blood progenitor and/or effector cells; and by massive deposition of collagen in the connective tissue of the skin and various internal organs.
 
Pathways In Pulmonary Arterial Hypertension PDF Print E-mail
Friday, 08 March 2013 00:09
It is well established that the endothelin, nitric oxide and prostacyclin pathways play an important role in the development of pulmonary arterial hypertension (PAH). Indeed, the therapeutic options currently available for the management of PAH all act on one of these mechanistic pathways. However, this is an exciting time for both clinicians and scientists, as increased understanding of the mechanisms involved in the pathogenesis and progression of PAH has resulted in the development of a number of novel therapeutic options.
 
Early Detection and Management of Pulmonary Arterial Hypertension PDF Print E-mail
Wednesday, 06 March 2013 16:03
The long-term prognosis for patients with pulmonary arterial hypertension (PAH) remains poor, despite advances in treatment options that have been made in the past few decades. Recent evidence suggests that World Health Organization functional class I or II patients have significantly better long-term survival rates than patients in higher functional classes, thus providing a rationale for earlier diagnosis and treatment of PAH. However, early diagnosis is challenging and there is frequently a delay between symptom onset and diagnosis.
 
Sexual Activity and Impairment in Women with Systemic Sclerosis Compared to Women from a General Population Sample PDF Print E-mail
Wednesday, 30 January 2013 23:58
Systemic sclerosis, or scleroderma, is a chronic, multisystem, connective tissue disorder characterized by abnormal fibrotic processes and excessive collagen production, which manifests itself in skin thickening and fibrosis of internal organs. Approximately 80% of SSc patients are women, with highest onset rates between ages 30–60. Common causes of disability include limitations in physical mobility, pain, fatigue, depressive symptoms, and body image distress from disfigurement.

In the general population, sexual activity and impairment rates are, among other factors, highly associated with age and marital status
 
Modulation of Fibrosis in Systemic Sclerosis by Nitric Oxide and Antioxidants PDF Print E-mail
Wednesday, 30 January 2013 23:48
Systemic sclerosis is a multisystem, connective tissue disease of unknown aetiology characterized by vascular dysfunction, autoimmunity, and enhanced fibroblast activity resulting in fibrosis of the skin, heart, and lungs, and ultimately internal organ failure, and death. One of the most important and early modulators of disease activity is thought to be oxidative stress.

Evidence suggests that the free radical nitric oxide, a key mediator of oxidative stress, can profoundly influence the early microvasculopathy, and possibly the ensuing fibrogenic response.
 
Survival In Systemic Sclerosis-Associated Pulmonary Arterial Hypertension PDF Print E-mail
Wednesday, 30 January 2013 23:27
Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc) and one of the leading causes of morbidity and mortality in this disease. Although several recent studies have suggested an improvement in the prognosis of SSc-PAH, especially since the availability of specific therapies, the overall survival at 3 years remains poor (around 50%) and worse than in patients with idiopathic PAH. Many studies have addressed the survival and prognostic factors in SSc-PAH.
 
Pulmonary Arterial Hypertension: Bridging The Present To The Future PDF Print E-mail
Wednesday, 30 January 2013 23:07
The past decade has witnessed extensive progress in basic and clinical research in the field of pulmonary hypertension (PH), a group of chronic conditions characterised by high pressure in the pulmonary circulation. National and international PH registries have achieved much to advance our understanding of the epidemiology, demographics, aetiology, clinical course, haemodynamics, disease management and treatment outcomes of PH. Therapies available to target the pathology of pulmonary arterial hypertension (PAH) have expanded considerably and more options are expected in the near future.
 
L-selectin and Skin Damage in Systemic Sclerosis PDF Print E-mail
Saturday, 15 September 2012 14:19
L-selectin ligands are induced on the endothelium of inflammatory sites. L-selectin expression on neutrophils and monocytes may mediate the primary adhesion of these cells at sites of inflammation by mediating the leukocyte-leukocyte interactions that facilitate their recruitment. L-selectin retains functional activity in its soluble form.

Levels of soluble L-selectin have been reported as both elevated and lowered in patients with systemic sclerosis (SSc).
 
The Role of Intravenous Immunoglobulin Preparations in the Treatment of Systemic Sclerosis PDF Print E-mail
Saturday, 16 June 2012 03:43
Marta Baleva and Krasimir Nikolov
Received 12 June 2011; Revised 28 August 2011; Accepted 28 August 2011

Scleroderma is progressive autoimmune disease associated with severe disability. The major underlying pathological process in Scleroderma is progressive development of fibrous tissue and obliteration of the microvasculature. Currently, there are no medical products for the treatment of Scleroderma that provide both sufficient immunosuppression and low-risk side safety profile with negligible side effects.

There are a large number of experimental data showing that intravenous immunoglobulin (IVIG) has multiple clinical and morphological effects. On the other hand, some authors report good effect of intravenous immune globulins in patients with Scleroderma. The less frequent side effects of IVIG in doses below or equal to 2 g/kg/month divided in 5 consecutive days make IVIG a promising treatment of choice in Scleroderma.
 
PDE-5 Inhibitors in Scleroderma Raynaud Phenomenon and Digital Ulcers PDF Print E-mail
Thursday, 03 May 2012 23:17
Systemic sclerosis- (SSc-) related vasculopathy, as manifested by Raynaud’s Phenomenon (RP) and digital ulcers (DUs), is associated with significant impairment of the quality of life and morbidity. The current vasoactive approach for SSc-RP, although employing vasodilators, is entirely off-label. PDE-5 inhibitors improve peripheral circulation, are well tolerated, and are widely used for various forms of constrictive vasculopathies.

This class of medications has become one of the first lines of treatment of SSc-RP and SSc-DUs among rheumatologists that routinely treat SSc patients.
 
Silicone and Scleroderma Revisited PDF Print E-mail
Thursday, 03 May 2012 23:09
Silicone, a synthetic polymer considered to be a biologically inert substance, is used in a multitude of medical products, the most publicly recognized of which are breast implants. Silicone breast implants have been in use since the early 1960s for cosmetic and reconstructive purposes, and reports of autoimmune disease-like syndromes began appearing in the medical literature soon thereafter. Over the previous year, silicone implants have been suggested as playing a role in a new syndrome that encompasses a wide array of immune-related manifestations, termed ASIA (‘Autoimmune Syndrome Induced by Adjuvant’).

Scleroderma, a relatively rare connective tissue disease with skin manifestations and systemic effects, has also been described in association with silicone implantation and rupture.
 
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