Video: Every Case Tells a Story| Webinar: ACR/CHEST ILD Guidelines in Practice

An official publication of the ACR and the ARP serving rheumatologists and rheumatology professionals

Systemic Sclerosis

The Latest Advances in Sjögren’s, Scleroderma, RA, Gout & More

  |  

ATLANTA—At the ACR/ARP 2019 Annual Meeting, several widely renowned experts across an array of specialty subjects provided a comprehensive and compelling review of advances in the understanding, diagnosis and treatment of a number of rheumatologic conditions. Sjögren’s Syndrome Frederick Vivino, MD, FACR, chief of rheumatology at Penn Presbyterian Medical Center and professor of clinical medicine…

FDA Approves Nintedanib for SSc-ILD, But Temper Your Expectations

Michael Putman, MD  |  

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…

Case Report: A Patient Develops Scleroderma Renal Crisis

Adria Madera-Acosta, MD, Teresa Sosenko, MD, & Diana Girnita, MD, PhD  |  

Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis. SRC occurs in 2–15% of patients with diffuse sclerosis and usually within the first five years from the time of diagnosis. Risk factors for SRC include, but are not limited to, early diagnosis, corticosteroid or cyclosporine use, and the presence of anti-RNA polymerase III…

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….

Light micrograph of a vascular lesion (center) caused by systemic sclerosis.

Case Report: Can Salt-&-Pepper Skin Mean Systemic Sclerosis?

Wesam Gouda, MBBCh, MSc; Abdelhafeez Moshrif, MBBCh, MSc, MD; Fatma H. El Nouby, MBBCh, MSc, MD; & Amal Fehr, MBBCh, MSc, MD  |  

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

Bryn Nelson, PhD  |  

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)

Elizabeth R. Volkmann, MD, MS, Michael D. Roth, MD, Donald P. Tashkin, MD, Cathie Spino, ScD, & Dinesh Khanna, MD, MS  |  

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…