News for Patients

Screening for PAH in Systemic Sclerosis: Does It Matter?

Author: Kevin Deane, MD
Date Published: February-2012
Source: Medscape Today

ulmonary arterial hypertension (PAH) is a leading cause of mortality in patients with systemic sclerosis (SSc).[1] Once commonly thought to be a late complication of SSc, support for PAH as an early manifestation of disease is growing. PAH is present within 5 years of the onset of the first non-Raynaud symptoms of SSc in approximately 50% of patients.

 

Emerging patterns of genetic overlap across autoimmune disorders.

Author: Richard-Miceli C, Criswell LA.
Date Published: January-2012
Source: Genome Medicine

Most of the recently identified autoimmunity loci are shared among multiple autoimmune diseases. The pattern of genetic association with autoimmune phenotypes varies, suggesting that certain subgroups of autoimmune diseases are likely to share etiological similarities and underlying mechanisms of disease.

 

An Evaluation of Long-Term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From REVEAL.

Author: Benza RL, Miller DP, Barst RJ, et al
Date Published: January-2012
Source: Chest

The Registry to EValuate Early And Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) was established to characterize the clinical course, treatment, and predictors of outcomes in patients with pulmonary arterial hypertension (PAH) in the US. To date, estimated survival based on time of patient enrollment has been established and reported.

 

Evidence of the contribution of the X chromosome to systemic sclerosis susceptibility: association with the functional IRAK1 196Phe/532Ser haplotype.

Author: Dieudé P, Bouaziz M, Guedj M, et al
Date Published: December-2011
Source: Arthritis & Rheumatism

Several autoimmune disorders, including systemic sclerosis (SSc), are characterized by a strong sex bias. To date, it is not known whether genes on the sex chromosomes influence SSc susceptibility. Recently, an IRAK1 haplotype that contains the 196Phe functional variant (rs1059702), located on Xq28, was found to confer susceptibility to systemic lupus erythematosus (SLE).

 

Mechanism, Potential Therapy for Scleroderma Lung Disease Uncovered

Author: NIAMS / Tourkina E, et al
Date Published: January-2012
Source: NIAMS Spotlight on Research 2012

Investigators, partially supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases, have found that a deficiency in the protein caveolin-1 (cav-1) is linked to the development of interstitial lung disease, the scarring of lung tissue that causes disability and death in people with scleroderma.

 

Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers

Author: Barnes, J.; Mayes, M.
Date Published: January-2012
Source: Current Opinion in Rheumatology

SSc disease occurrence data are now available for Argentina, Taiwan, and India and continue to show wide variation across geographic regions. The survival rate is negatively impacted by older age of onset, male sex, scleroderma renal crisis, pulmonary fibrosis, pulmonary arterial hypertension, cancer, and antitopoisomerase and anti-U1 antibodies.

 

Work productivity in scleroderma: Analysis from the University of California, Los Angeles scleroderma quality of life study

Author: M. Singh, P. Clements, D. Furst, et al
Date Published: January-2012
Source: Arthritis & Rheumatism

One hundred sixty-two patients completed the Work Productivity Survey. Patients indicated whether or not they were employed outside of the home, how many days per month they missed work (employment or household work) due to SSc, and how many days per month productivity was decreased by ≥50%.

 

Haemopoietic stem-cell transplantation for systemic sclerosis

Author: K. Sullivan, F. Wigley, C. Denton, J. van Laar, D. Furst
Date Published: January-2012
Source: The Lancet

Richard Burt and colleagues (Aug 6, p 498)1 propose, on the basis of a single-centre trial of 19 patients, that autologous haemopoietic stem-cell transplantation (HSCT) might be the new standard of care for diffuse systemic sclerosis. Although the study strongly supports the use of immunosuppression in systemic sclerosis, several limitations are to be noted.

 

Emerging targeted therapies in scleroderma lung and skin fibrosis.

Author: Maurer B, Distler O.
Date Published: January-2012
Source: Clinical Rheumatology

Systemic sclerosis (SSc) is a multisystemic fibrotic disorder that affects the skin and internal organs. Despite an improved outcome probably reflecting a better management of disease complications, morbidity and mortality remain higher than those of patients with other connective tissue diseases.

 

32 Million Americans Have Autoantibodies That Target Their Own Tissues

Date Published: January-2012
Source: NIEHS

More than 32 million people in the United States have autoantibodies, which are proteins made by the immune system that target the body’s tissues and define a condition known as autoimmunity, a study shows.

 

Methotrexate and Corticosteroids in the Treatment of Localized Scleroderma: A Standardized Prospective Longitudinal Single-center Study

Author: Kathryn S. Torok and Thaschawee Arkachaisri
Date Published: January-2012
Source: The Journal of Rheumatology

To evaluate the effectiveness of a uniform single-center treatment protocol composed of high-dose methotrexate (MTX) and oral corticosteroids in a pediatric localized scleroderma (LS) cohort.

 

New NIH center translates research into real-world use

Author: J. Lee
Date Published: January-2012
Source: FederalNewsRadio.com

This month the National Institutes of Health created the National Center for Advancing Translational Sciences (NCATS) to bridge the gap between scientific research and usable drugs or medical devices. The reorganization was the biggest at NIH in a decade, according to ScienceInsider Magazine.

 

SSc in 2011: From mechanisms to medicines

Author: L. Mouthon
Date Published: January-2012
Source: Nature Reviews Rheumatology

Findings from ongoing studies of imatinib in systemic sclerosis (SSc) were eagerly awaited in 2011, but results from these clinical trials have so far been disappointing. However, progress in the understanding of the mechanisms that underlie SSc pathogenesis could provide clues to novel targets for 2012 and beyond.

 

Pulmonary hypertension in kids differs from adult PAH

Author: L. Nainggolan
Date Published: January-2012
Source: Heartwire

A new registry of children with pulmonary hypertension is helping to differentiate the pediatric population with this disease from the adult one and will hopefully aid in improvements in the diagnosis and treatment of this condition in kids, say the authors.

 

Top 10 Medical Research Trends to Watch in 2012

Author: M. Anderson
Date Published: January-2012
Source: Huffington Post

This year, the National Institutes of Health will establish its new National Center for Advancing Translational Sciences (NCATS), created to speed the translation of basic discoveries into therapies that will improve public health.

 

Unraveling the genetic component of systemic sclerosis.

Author: Martín JE, Bossini-Castillo L, Martín J.
Date Published: January-2012
Source: Human Genetics

Systemic sclerosis (SSc) is a severe connective tissue disorder characterized by extensive fibrosis, vascular damage, and autoimmune events. During the last years, the number of genetic markers convincingly associated with SSc has exponentially increased.

 

Preterm Delivery a Risk in Systemic Sclerosis

Author: H. Splete
Date Published: January-2012
Source: MedConnect

Many women with systemic sclerosis can have successful pregnancies, but the rates of preterm birth, low birth weight, and intrauterine growth restriction are approximately twice as high in these women compared to the general population of pregnant women, based on data from 109 pregnancies in 99 women with systemic sclerosis.

 

An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma.

Author: Coghlan JG, Schrieber B.
Date Published: January-2012
Source: Current Rheumatology Reports

Pulmonary arterial hypertension associated with scleroderma (SScPAH) is a debilitating, highly lethal condition that responds to an array of therapies. Quality of life and prognosis are substantially improved by treatment, and early diagnosis and treatment are associated with improved outcomes.

 

Understanding fibrosis in systemic sclerosis: shifting paradigms, emerging opportunities

Author: S. Bhattacharyya, J. Wei & J. Varga
Date Published: January-2012
Source: Nature Reviews Rheumatology

Fibrosis in multiple organs is a prominent pathological finding and distinguishing hallmark of systemic sclerosis (SSc). Findings during the past 5 years have contributed to a more complete understanding of the complex cellular and molecular underpinning of fibrosis in SSc.

 

The impact of cardio-pulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies.

Author: Komócsi A, Vorobcsuk A, Faludi R, et al
Date Published: January-2012
Source: Rheumatology (Oxford)

Internal organ involvement reduces the life expectancy of SSc patients. Cardiopulmonary manifestations are currently the primary cause of death.