Scleroderma

Australia is in a state of ‘clinical equipoise’ over whether to anticoagulate systemic sclerosis patients with pulmonary hypertension, experts say. And with observational studies showing the treatment is associated with a  fivefold reduction in mortality, the issue demanded a prompt resolution, the team of rheumatologists wrote in this week’s Internal Medicine Journal.

Describing the issue as one of the “most contentious” in the management of patients with connective tissue disease, the doctors said anticoagulation was currently not regarded as standard care in these

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.

Men with autoimmune diseases have a higher incidence of prostate cancer (PCa) than those without those diseases, according to study findings presented at the American Urological Association annual meeting.

Using the National Inpatient Sample database, researchers obtained data on 189,290 men with a history of autoimmune disease—multiple sclerosis (MS), systemic lupus erythematosus (SLE), systemic sclerosis, psoriasis, Sjogren's syndrome, discoid lupus erythematosus (DLE), or rheumatoid arthritis (RA)—and a subsequent diagnosis of PCa.

The University of Michigan Health System is widely known for medical innovation — from the research laboratory to the patient’s bedside — but in partnership with dedicated donors, the institution is also innovating the way it raises money to accelerate groundbreaking research.

With the help of local philanthropists Jon and Lisa Rye, whose close family member was diagnosed with the debilitating skin disease Scleroderma, UMHS is reaching out to the Scleroderma community to help fund promising new research underway in the U-M Division of Rheumatology.

Systemic sclerosis (SSc), also known as Scleroderma, is a rare autoimmune connective tissue disorder that's difficult to treat. However, thanks to new research at Dartmouth's Geisel School of Medicine and Northwestern University's Feinberg School of Medicine, doctors may be able to treat some patients more effectively.

Characterized by thickening of the skin, SSc can also cause significant complications in the joints and internal organs—particularly the esophagus, lower gastrointestinal tract, lungs, heart and kidneys. There is no cure—and the one drug commonly used to treat the disease, mycophenolate mofetile (MMF) does not work for all patients. In the absence of a biomarker to inform therapeutic medical decisions, patients are exposed to ineffective and potentially toxic medications.

Scleroderma is a rare, autoimmune, connective tissue disease characterized by the overproduction of collagen, which results in the thickening and hardening of the underlying connective tissues which support the skin, blood vessels, muscles, and internal organs such as the heart, lungs, and kidneys. It can limit a patient’s range of motion and ability to perform simple daily tasks due to fatigue, and the pain, swelling, and inflammation of the joints. With organ involvement, life can become particularly more challenging and life-threatening. There is no known cure for Scleroderma.

Using the VivoSight OCT scanner, manufactured in the UK by Michelson Diagnostics Ltd, scientists at the Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds have developed the first quantitative imaging biomarker for skin involvement in Scleroderma.

Scleroderma or Systemic Sclerosis (SSc) is a serious type of autoimmune disorder affecting approximately 1 in 10,000 people. It is a progressive disease that involves skin and internal organs by determining fibrosis, vasculopathy and immune system activation.

Patients with diffuse systemic sclerosis (SSc) and no clinical evidence of cardiovascular disease (CVD) may, in fact, have subclinical CVD and atherosclerosis, and it is detectable. This is the conclusion of a small pilot study from Italy, which showed that elevated plasma levels of asymmetric dimethylarginine (ADMA), an endogenous nitric oxide synthase inhibitor important in the atherosclerotic process, may be a unique indicator of cardiovascular risk in systemic sclerosis (SSc).

The immune-inflammatory response that injures vascular endothelial cells in

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.

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.

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.

Researchers in Italy evaluated 65 patients with Juvenile Localized Scleroderma (JLS; or morphea) previously enrolled in a double blind, randomized control trial and treated for the first 3 months with oral methotrexate (MTX; 15 mg/m2 weekly) and prednisone (1 mg/kg daily, maximum 50 mg). Clinical evaluation, infrared thermography and computerized skin scores were used to evaluate lesions.

Patients were defined as responders if they satisfied criteria including no new lesions, skin score rate less than 1 and a decrease in lesion temperature by at least 10% compared with baseline.

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

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.