For Patients

If you have scleroderma, you are not alone. The scleroderma community is made up of tens of thousands of patients and their loved ones worldwide. The SRF is here to help.

The first time many people hear about scleroderma is when they, a family member or friend are diagnosed with the disease. Scleroderma is a complex and surprisingly widespread illness, affecting as many people as more commonly recognized diseases such as multiple sclerosis and muscular dystrophy.

In addition to funding the most promising research aimed at improved therapies and a cure, the Scleroderma Research Foundation provides information that may help scleroderma patients better understand their disorder and more effectively manage its symptoms.

To learn more about the various forms and subtypes of scleroderma, please click here. This section of the Scleroderma Research Foundation’s Web site provides information for patients to educate themselves, as well as their caretakers and loved ones, about this serious disease.

Please remember, information provided on this Website and others is intended as a guide. Specific medical advice can only be provided by your health care professional.

 
 

Research News

Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities

Author: John Varga, Maria Trojanowska, Masataka Kuwana
Date Published: July-2017
Source: Journal of Scleroderma and Related Disorders

Systemic sclerosis (SSc) is a complex disease characterized by early microvascular abnormalities, immune dysregulation and chronic inflammation, and subsequent fibrosis of the skin and internal organs. Excessive fibrosis, distinguishing hallmark of SSc, is the end result of a complex series of interlinked vascular injury and immune activation, and represents a maladaptive repair process. Activated vascular, epithelial, and immune cells generate pro-fibrotic cytokines, chemokines, growth factors, lipid mediators, autoantibodies, and reactive oxygen species. These paracrine and autocrine cues in turn induce activation, differentiation, and survival of mesenchymal cells, ensuing tissue fibrosis through increased collagen synthesis, matrix deposition, tissue rigidity and remodeling, and vascular rarefaction.

The mighty fibroblast and its utility in scleroderma research

Author: Sara M. Garrett, DeAnna Baker Frost, Carol Feghali-Bostwick
Date Published: May-2017
Source: Journal of Scleroderma and Related Disorders

Fibroblasts are the effector cells of fibrosis characteristic of systemic sclerosis (SSc, scleroderma) and other fibrosing conditions. The excess production of extracellular matrix (ECM) proteins is the hallmark of fibrosis in different organs, such as skin and lung. Experiments designed to assess the pro-fibrotic capacity of factors, their signaling pathways, and potential inhibitors of their effects that are conducted in fibroblasts have paved the way for planning clinical trials in SSc. As such, fibroblasts have proven to be valuable tools in the search for effective anti-fibrotic therapies for fibrosis. Herein we highlight the characteristics of fibroblasts, their role in the etiology of fibrosis, utility in experimental assays, and contribution to drug development and clinical trials in SSc.This article is available for you to download for free for 1 week until 2nd August 2017.

Systemic Sclerosis Linked With Altered Gut Microbiome

Author: Gregory M. Weiss, MD
Date Published: July-2017
Source: Rheumatology Network

The numbers of Bacteroidetes bacteria in the GI tracts of 2 separate cohorts of patients with systemic sclerosis were significantly reduced when compared with healthy controls. American patients with systemic sclerosis had more extensive alterations in their intestinal microbiota than those in a Norwegian cohort. An abundance of Prevotella species was associated with moderate-to-severe GI symptoms in patients with systemic sclerosis. Clostridium species abundance was associated with low GI symptom severity, and Lactobacillus with none-to-mild constipation.

Biomarkers Predict Digital Vascular Events in Scleroderma

Author: Gregory M. Weiss, MD
Date Published: July-2017
Source: Rheumatology Network

The likelihood of digital vascular pits, ulcers, or gangrene is increased in patients with scleroderma who are double-positive for anti-interferon-inducible protein 16 and anticentromere autoantibodies. Particularly high anti-interferon-inducible protein 16 levels were found in patients with scleroderma who had active ischemic ulcers or gangrene. Measuring anti-interferon-inducible protein 16 levels in patients with scleroderma and anticentromere antibody positivity may help stratify those at high risk for significant digital vascular events.

Mortality risk prediction in scleroderma-related interstitial lung disease: the SADL model

Author: Julie Morisset, MD; Eric Vittinghoff, PhD; Brett M. Elicker, MD; Xiaowen Hu, MD; Stephanie Le, MD; Jay H. Ryu, MD; Kirk D. Jones, MD; Anna Haemel, MD; Jeffrey A. Golden, MD; Francesco Boin, MD; Brett Ley, MD; Paul J. Wolters, MD; Talmadge E. King, Jr., MD; Harold R. Collard, MD FCCP; Joyce S. Lee, MD
Date Published: June-2017
Source: Chest Journal

Rationale Interstitial lung disease (ILD) is an important cause of morbidity and mortality in scleroderma (Scl). Risk prediction and prognostication in Scl-ILD patients is challenging because of heterogeneity in the disease course.

News for Patients

Uptravi Slows Progression of Pulmonary Hypertension Stemming from Connective Tissue Disease, Study Finds

Author: Iqra Mumal
Date Published: August-2017
Source: Pulmonary Hypertension News

Actelion’s Uptravi (selexipag) delays the progression of pulmonary arterial hypertension that stems from connective tissue disease, a follow-up analysis of Phase 3 clinical trial findings indicates. People with PAH-CTD also tolerate the therapy well, according to the study. The PAH part of PAH-CTD refers to high blood pressure in the lungs’ arteries.

Prucalopride Can Help Improve Gastrointestinal Symptoms in SSc Patients, Study Shows

Author: Alice Melao
Date Published: August-2017
Source: Scleroderma News

Prucalopride, marketed under the name Resolor in Europe and Resotran in Canada, can safely manage mild to severe gastrointestinal (GI) symptoms associated with systemic sclerosis (SSc), including bowel movement and transit, reflux disease, and bloating, finds a study published in the journal Arthritis Research and Therapy. Doctors have proposed using medicines to improve prokinetics, or motility of the GI tract, in treating SSc patients. But the lack of proper clinical trials demonstrating the therapeutic potential of such drugs in the SSc population discouraged their use in clinics.

How to Fuel Engaged Patient Dialogue

Author: Carina Stanton
Date Published: July-2017
Source: The Rheumatologist

In the average 10–15 minutes a rheumatologist has to spend with a patient during a clinic visit, limited time exists for extensive dialogue. Yet, taking the time to delve a little deeper by asking a patient questions in order to understand and address concerns could make a big difference in a patient’s care.

“Too often, a patient may feel [too] intimidated to ask a question to clarify a term we use or may fear embarrassment [and avoid] bringing up a concern that can be related to their condition, such as intimacy issues,” notes Ara Dikranian, MD, a rheumatologist with the Cabrillo Center for Rheumatology in El Cajon, Calif.

Use of intravenous epoprostenol as a treatment for the digital vasculopathy associated with the scleroderma spectrum of diseases

Author: Law, S.,Farber, H., Simms, R. 
Date Published: August-2017
Source: Journal of Scleroderma and Related Disorders

Intravenous prostanoid therapy is recommended for severe systemic sclerosis-related digital vasculopathy. The evidence supporting this recommendation is limited. The aim of this study was to evaluate the safety and efficacy of treating scleroderma spectrum digital vasculopathy with intravenous epoprostenol.

5 Tips for Managing Raynaud’s Syndrome Through the Summer

Author: Wendy Henderson
Date Published: July-2017
Source: Scleroderma News

You may think the summer months offer some respite to those who suffer from Raynaud’s syndrome but the warmer months can bring their own set of problems. Many offices, shopping malls, and other public spaces turn on the air conditioning in the warmer weather, which is great for helping you cool down, but many places have the AC turned up so high that it brings on symptoms of Raynaud’s.

Ways to Give

There are many ways that you can support the work of the Scleroderma Research Foundation. We are grateful for your commitment to helping the SRF fund research that will result in improved therapies and, ultimately, a cure.

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