In a new study, Spiera et al. assessed the safety and efficacy of lenabasum, a synthetic, orally administered agonist of cannabinoid receptor 2 that modulates the endocannabinoid system to activate the resolution phase of innate immune responses, in diffuse cutaneous systemic sclerosis…
Systemic Sclerosis Patients May Benefit from Targeted Stroke Screening
Patients with systemic sclerosis (SSc) may benefit from targeted stroke screening or prevention therapies. A recent study revealed SSc may be independently associated with stroke, finding the risk of stroke was 20–30% higher in SSc patients than healthy controls…
FDA Approves Nintedanib for SSc-ILD, But Temper Your Expectations
The U.S. Food & Drug Administration (FDA) approved nintedanib for systemic sclerosis associated interstitial lung disease (SSc-ILD) on Sept. 6 after a randomized, controlled trial (SENSCIS) demonstrated significant benefit against placebo.1 At a cost of $96,000 per year, treatment reduced the adjusted annual rate of change in forced vital capacity (FVC) from –93.3 mL in…
Nailfold Videocapillaroscopy Provides a Window into Inflammatory Myopathies
A recent study investigated the possible differences in nailfold videocapillaroscopy in four types of inflammatory myopathies. Researchers observed giant capillaries, disorganization and major capillary loss in dermatomyositis and overlap myositis patients, finding dermatomyositis and overlap myositis imaging was different from that of antisynthetase syndrome and immune-mediated necrotizing myopathy…
Can REVEAL Tool Predict Survival in SSc-Related Pulmonary Arterial Hypertension?
A prognostic tool developed to predict survival in patients with various forms of pulmonary arterial hypertension (PAH) is fairly accurate in predicting survival outcomes for many patients with PAH related to systemic sclerosis (SSc-PAH), according to a new study. However, the prognostic accuracy is less reliable for SSc-PAH patients with the highest risk of death….
Case Report: Can Salt-&-Pepper Skin Mean Systemic Sclerosis?
Systemic sclerosis (SSc) is a multi-system connective tissue disease in which skin and internal organ fibrosis are associated with an obliterative micro-vasculopathy and a degree of inflammation.1 Patients often report it takes one to three years from the appearance of the first signs and symptoms before they receive a diagnosis. The signs and symptoms of…
Study Probes New Gene Therapy for Severe, Localized Scleroderma (Morphea)
In September 2018, the U.S. Food & Drug Administration (FDA) granted fast-track status to FCX‑013, a gene therapy product developed to treat moderate to severe localized scleroderma (morphea). Previously, the treatment received an orphan drug designation for localized scleroderma, as well as a rare pediatric disease designation. Phase 1 and 2 studies will assess safety…
New Studies Identify Possible Markers of Early Systemic Sclerosis
Although the true cause of systemic sclerosis (SSc), or scleroderma, remains unknown, researchers have made progress in detecting the autoimmune disease’s early presence. Beyond the physiological signs of Raynaud’s phenomenon, a capillaroscopy can detect alterations in microcirculation and lab tests can confirm the presence of telltale autoantibodies, such as anti-topoisomerase 1, anti-centromere and anti-RNA polymerase…
Lessons Learned from Two Scleroderma Lung Studies (Plus a Third That’s Recruiting Sites)
Historically, the early approach for treating interstitial lung disease (ILD) due to systemic sclerosis (SSc) involved immunosuppressant therapy, primarily with cytotoxic agents.1 Glucocorticoids in combination with another immunosuppressant agent, such as oral azathioprine or cyclophosphamide, were often used to treat patients with severe, progressive SSc-ILD.2 However, direct evidence to support this therapeutic approach was lacking…
Recent Trials Investigated Targeted Therapies for Systemic Sclerosis
SNOWMASS VILLAGE, COLO.—Data from three recent trials in systemic sclerosis (scleroderma) provide information on a number of important issues related to screening and treatment. First presented at the 2018 ACR/ARHP Annual Meeting, the phase 2/3 trials assessed the safety and efficacy of targeted agents to treat patients with systemic sclerosis.1-3 In a follow-up presentation at…