About 5 percent of Americans have Raynaud’s syndrome, in which spasms of the arteries in the hands and feet, usually caused by cold or stress, temporarily cut off blood flow. This can cause the fingers and toes to change color, said Dr. Gordon — white and blue in lighter skin, or more yellow and purple in darker skin. While Raynaud’s is generally harmless, it can occasionally be caused by serious autoimmune diseases like lupus or scleroderma, she added.

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Many rheumatologists are likely familiar with avacopan, but are not used to prescribing it, explained Dr. Lally. 

"Rituximab was approved for GPA (granulomatosis with polyangiitis) and MPA (microscopic polyangiitis) a decade ago at this point. It was a drug that we as rheumatologists were used to using. We used it for other indications. Avacopan is a totally new drug, a new mechanism of action, so there's not a lot of extractable data that we have in terms of comfort with the drug, and so I think that's one of the biggest hurdles," she said.

Read the full article at medscape.com. 

According to Dr. Spiera, although lenabasum failed to meet its efficacy endpoint in a trial of patients with diffuse cutaneous systemic sclerosis, a post-hoc analysis suggests an impact on forced vital capacity (FVC).

To examine the efficacy, safety and tolerability of lenabasum compared with placebo, Dr. Spiera and colleagues conducted the double-blind, randomized, phase-3 RESOLVE-1 trial. Study participants were allowed to receive background immunosuppressive therapies because it “reflects real-world clinical practice in the treatment of systemic sclerosis,” Dr. Spiera explained.

The researchers found that mycophenolate also impacted outcomes. Dr. Spiera noted, “Patients who entered the trial on mycophenolate were more likely to have a more favorable response with regard to FVC, whereas other features, including the region they came from or disease duration did not. Baseline FVC also came up statistically significant in this model.”

Dr. Spiera concluded, “Evidence for an effect of lenabasum on forced vital capacity [FVC] was suggested by post-hoc analyses that considered the effect of background immunosuppressive therapies on outcomes.”

He added, “Results from the post-hoc analyses will require confirmation in additional studies to determine the potential of lenabasum for treating patients with systemic sclerosis. Treatment with lenabasum was safe and well-tolerated in this study.”

Read the full article at Healio.com.

Additional coverage: Medscape.com, MDedge.com.

HSS rheumatologist Kimberly Showalter, MD received a three-year grant to determine if fibroblasts - a key cell type in scleroderma that produces collagen — can help predict clinical improvement in response to treatment. Ultimately, this may help offer treatment to people more likely to benefit, and spare from toxic effects those unlikely to improve.

This coverage originally appeared in Sclerodermanews.com. The original article is no longer available online.

HSS rheumatologist Robert F. Spiera, MD who was not involved in the study, provided comments on the results, noting, "This was a really interesting study looking at a novel strategy for patients with early diffuse systemic sclerosis, which is a real challenge in terms of identifying effective therapies.” He cited, “This was a trial design that is very impressive to me in the sense that they did what we do in the real world, which was to allow background therapy.” Dr. Spiera added, "Here they were adding the experimental treatment to standard of care therapy, meaning that all patients were able to receive such therapies, which could in theory make it harder to demonstrate that the study medication was having a beneficial effect."

While the study was limited by its brief duration, the impact on skin met the prespecified endpoint, which is the first time that this has been achieved in a Phase II study in diffuse cutaneous systemic sclerosis. "It definitely showed signals of efficacy and was acceptable in terms of safety. This would warrant a further look in a large Phase III clinical trial," he concluded.

Read the full article at MedPageToday.com.

While a diagnosis of scleroderma certainly brings challenges to pregnancy, proper planning and prenatal care can help ensure the best possible outcomes for the mother and baby, said Dr. Sammaritano. When planning for pregnancy, it is important to wait at least three years after receiving a diagnosis to become pregnant, as the course of the disease can be unpredictable and flares are more likely. Dr. Sammaritano recommended discussing any family planning issues with a rheumatologist early on, as pre-planning will allow the patient and their physician to make sure disease activity is low while on pregnancy-compatible medications. “This process can take some time, since changing a medication means giving it several months to make sure that it works and doesn’t cause side effects,” she explained.

Read the full article at Arthritis.org.

“Esophageal involvement in scleroderma is incredibly common, but some of that depends on how it is defined and how aggressively it is searched for,” explained Dr. Spiera. “About half of patients with scleroderma have GERD [gastroesophageal reflux disease] or dysphagia (difficulty swallowing),” he added.

Treating GERD and other GI issues begin with lifestyle changes, such as eating smaller and more frequent meals throughout the day. “Since your motility is diminished, it is hard for the GI tract to handle a larger volume load,” noted Dr. Spiera. “You don’t, however, want to restrict total calorie consumption.” Dr. Spiera also advised not to eat within a few hours of bedtime. “Since gastric emptying can be delayed, eating earlier can be helpful as more of the food can move down the GI tract and enter the small intestine or beyond by the time you go to bed,” he said. Elevating the head of your bed a few inches can also help. “This helps use gravity to assist food moving along the GI tract, in particular the esophagus,” cited Dr. Spiera.

Read the article at Creakyjoints.org.

SRC is characterized by sudden hypertension (high blood pressure) and rapidly progressive renal failure, typically without previous significant kidney disease. SRC can also involve hypertensive encephalopathy (brain disfunction due to high blood pressure), congestive heart failure, and/or microangiopathic hemolytic anemia (loss of red blood cells). Dr. Spiera explained an early diagnosis of scleroderma is critical to reduce the risk of complications like SRC. “You have to be vigilant and take this condition seriously upfront,” he said. “About 10 percent of people with scleroderma renal crisis will still have normal blood pressure, though their blood pressure levels are elevated relative to their own baseline,” cited Dr. Spiera. “Patients who have hypertension often have some protein in the urine,” he added.

Patients with scleroderma will have their blood tested for certain antibodies, which can help confirm a diagnosis with SRC. “Checking blood levels of renin can be helpful,” noted Dr. Spiera, the enzyme that helps control your blood pressure, and is produced by cells in the kidneys. Treatment of SRC aims to control blood pressure with angiotensin-converting enzyme inhibitors (ACEIs). “You need to treat aggressively with ACEIs, pushing for the highest dose that’s tolerable by your blood pressure,” said Dr. Spiera.

Read the article at Creakyjoints.org.