News for Patients


Clinical Trial Enrolling - Rituximab for Treatment of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH)

Date Published: August-2017

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a serious, life-threatening manifestation of systemic sclerosis (SSc), an autoimmune disease of the connective tissue characterized by scarring (fibrosis) and atrophy of the skin, joints and tendons, skeletal muscles, and internal organs, and immunological disturbances. One-year survival for patients with SSc-PAH ranges from 50-81%. There is currently no cure for SSc-PAH and treatment is limited to vasodilator therapy used in all forms of PAH. In recent studies, immunotherapy was shown to be effective in treating SSc-interstitial lung disease, another serious, life-threatening manifestation of SSc. In addition, there are compelling pre-clinical data and anecdotal clinical reports that suggest modulation of the immune system may be an effective strategy for treating SSc-PAH. To test this approach, this trial will determine if rituximab, an immunotherapy, has a marked beneficial effect on clinical disease progression, with minimal toxicity, in patients with SSc-PAH when compared to placebo.

 

Phase 3 Trial of Actemra for Systemic Sclerosis Completes Enrollment

Author: Genentech
Date Published: August-2017
Source: Healio

This week, Genentech announced its phase 3 trial of Actemra for patients with systemic sclerosis has completed enrollment. The study will compare the drug’s safety and efficacy with placebo in patients with systemic sclerosis, according to a press release.

 

5 Ways Scleroderma Can Affect Your Eyes

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

Because scleroderma is an autoimmune disease which affects connective tissue, symptoms and complications can appear in any part of the body, including the eyes. This article includes a list of some of the more common eye complications experienced by people living with scleroderma.

 

8 Tips for Exercising When You Have Scleroderma

Author: Marta Duarte
Date Published: August-2017
Source: Scleroderma News

Balance your sessions. Even if you’re taking things slowly, if any of your joints feel painful for more than two hours after you workout, it’s recommended that you make the next exercise session less intense than the previous one. Slow down when necessary.

Enthusiasm is very good (and hard to come by most days) but if you feel out of breath or in more pain than you’re used to, slow down.

 

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.

 

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.

 

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.

 

More Than Systemic Sclerosis - The Inside Story

Author: Boehringer Ingelheim
Date Published: June-2017
Source: Boehringer Ingelheim

More than Systemic Sclerosis: The Inside Story’ is a collection of portraits showcasing the unique and inspiring stories of people living with systemic sclerosis, also known as scleroderma. Launched to coincide with World Scleroderma Day 2017 (29 June), this global initiative highlights the importance of understanding the ‘inside story’ of each individual living with systemic sclerosis. The initiative also aims to increase awareness of the potential serious consequences when fibrosis develops in vital organs such as the lungs. The new photography and film gallery features eight patients from seven countries, each telling their story about living with this rare condition.

 

Highland Park writer casts light on scleroderma

Author: Karen Berkowitz
Date Published: June-2017
Source: Chicago Tribune

Lisa Goodman-Helfand believes her childhood would have been less emotionally painful had she been told the gravity of her scleroderma diagnosis from the start. When she was 10, a series of tests at a Chicago pediatric hospital confirmed what a dermatologist had suspected. She had scleroderma, a potentially fatal disease of the autoimmune system characterized by a thickening and hardening of the skin.

 

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.

 

Galectin’s Candidate Therapy GR-MD-02 Patent Extended to Cover Several Illnesses Including Systemic Sclerosis

Author: Alica Melao
Date Published: June-2017
Source: Scleroderma News

The U.S. Patent and Trademark Office has granted Galectin Therapeutics a new patent that extends coverage of its candidate therapy GR-MD-02 to treat systemic sclerosis and other diseases in which high levels of the inducible nitric oxide synthase (or iNOS) enzyme causes inflammation. “With this patent extending claims to a wide-range of diseases with an inflammatory response, we now have a broad range of patent coverage both for diseases in which we currently have developmental programs, as well as, potential areas of future investigation,” Dr. Peter G. Traber, Galectin’s CEO and chief medical officer, said in a company press release.

 

10 Tips for a Healthy Recovery Following a Lung Transplant

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

For some chronic lung disease patients, their lung function declines so much that they need to have a lung transplant. However, it’s a complicated procedure and recovery can be slow. To ensure you have the healthiest recovery possible, you’ll need to adhere to a few basic rules, according to the Cleveland Clinic and the Cystic Fibrosis Foundation. MORE: As the weather turns warmer, many of us will be thinking about vacations and traveling. Here’s some advice for traveling with a lung disease.

 

Phase 2 Trial Results Presented of Corbus’ Anabasum as Treatment for Systemic Sclerosis

Author: Joana Fernandes, PhD
Date Published: June-2017
Source: Scleroderma News

Results of a Phase 2 clinical trial (NCT02465437) evaluating the effects of Corbus Pharmaceuticals’ anabasum (formerly known as Resunab or JBT-101) support this treatment as a potential option for patients with diffuse cutaneous systemic sclerosis. The study showed that treatment with anabasum affects the activity of nearly 1,940 genes associated with different mechanisms, including molecules associated with inflammation, skin regulation and structure, and fat metabolism.

 

#SclerodermaAwarenessMonth: Nicola Whitehill Profiles Scleroderma Patients From Around the World

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

One of our resident columnists, Nicola Whitehill, has been using Scleroderma Awareness Month to profile some of the people who live with the disease from around the world. MORE: Find out how you can support Scleroderma Awareness Month here. Writing on her own personal blog Raynauds Scleroderma Awareness, Nicola shares a rare view into the lives of those living with scleroderma. Nicola was diagnosed with scleroderma in 1997 at the age of 24 and was given just 15 months to live. Two decades later, she’s a proud advocate and activist for the disease.

 

Rheumatologist Calls for More Screening of Scleroderma Patients for Pulmonary Hypertension

Author: Joana Fernandes, PhD
Date Published: June-2017
Source: Scleroderma News

Many systemic sclerosis patients develop pulmonary arterial hypertension, but not all who are suspected of having PAH are referred for testing, according to rheumatologist Janet Pope. She called for more commitment to screening in an editorial in Arthritis Research & Therapy. The piece was titled “Pulmonary arterial hypertension in scleroderma: care gaps in screening.” PAH is a lung disease marked by high blood pressure in pulmonary arteries. It can lead to heart failure and death.

 

Scleroderma patients face barriers to treatment, discrimination at hands of insurers

Author: Chronic Disease Coalition
Date Published: June-2017
Source: Chronic Disease Coalition

Patient discrimination – from denying patients’ access to use charitable assistance to pay their bills to outright denial of coverage for lifesaving medical treatment – is a real threat for many people living with chronic and life-threatening conditions. And for people living with scleroderma, their story is sadly no different.

 

EULAR Update Targets Organ Involvement in Systemic Sclerosis

Author: Diana Swift
Date Published: May-2017
Source: MedPage Today

In a systematic review–based update of its 2009 recommendations, the European League Against Rheumatism (EULAR) has added two new therapeutic statements addressing organ complications in systemic sclerosis (SSC). These include Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, skin and lung disease, scleroderma renal crisis, and gastrointestinal involvement.

 

If the Shoe Fits … It’s a Major Feat!

Author: Lisa Goodman-Helfand
Date Published: March-2017
Source: Scleroderma News

Buying a new pair of shoes used to be one of my favorite things to do. The unsightly red spots, telangiectasia, which are generously sprinkled all over my body, limit the clothing I choose to wear. Anything backless, strapless, sleeveless, scoop-necked, or low-cut is immediately ruled out. Being that I am not by nature a conservative person, or Amish, having scleroderma has sort of sucked the joy out of shopping for clothes. But with shoes, the sky was the limit.

Though scleroderma impacted nearly every aspect of my appearance since my diagnosis at age 10, it had thankfully left my feet alone. High heels, platform shoes, open-toed sandals, flip-flops … no shoe was out of bounds for me. Aside from adhering to a reasonable budget, nothing stood between me and whatever shoes my heart desired.

 

Pneumocystis jiroveci pneumonia is a rare but serious complication for patients with rheumatic diseases

Author: Erika Darrah
Date Published: March-2017
Source: Johns Hopkins Medicine

A research team from the Johns Hopkins Division of Rheumatology lead by Christopher Mecoli, M.D., M.H.S, studied medical records of patients with rheumatic diseases who were admitted to the Johns Hopkins Hospital for pneumocystis jiroveci pneumonia (PJP) infection over a 20-year period (1996-2015). PJP is an uncommon but severe lung infection that can occur in patients with rheumatic disease, in particular in those who are taking medications that suppress the immune system.

 

Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS)

Author: A. Herrick, X. Pan, S. Peytrignet, M. Lunt, R. Hesselstrand, L. Mouthon, A. Silman, E. Brown, L. Czirják, J. Distler, O. Distler, et al
Date Published: February-2017
Source: Annals of the Rheumatic Diseases

Objectives The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches.

Methods This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or ‘no immunosuppressant’. Patients were assessed three-monthly for up to 24 months. The primary outcome was the change in modified Rodnan skin score (mRSS). Confounding by indication at baseline was accounted for using inverse probability of treatment (IPT) weights. As a secondary outcome, an IPT-weighted Cox model was used to test for differences in survival.