Scleromyxedema is a primary cutaneous mucinosis characterized by a diffuse and generalized papular skin eruption of mucinous deposits throughout the upper dermis. In addition to dermatologic manifestations, scleromyxedema may involve the cardiopulmonary, gastrointestinal, renal and nervous systems. Dermato-neuro syndrome (DNS) is a rare, severe neurologic complication of scleromyxedema.1,2 The pathogenesis of DNS is unknown, but…
No one-size-fits-all approach exists for the care and treatment of patients with systemic sclerosis (SSc) and SSc with pulmonary involvement. Here, experts discuss some best clinical practices for these patients.
McMahan et al. examined how abnormal gastrointestinal (GI) transit may contribute to GI severity and symptoms in patients with systemic sclerosis (SSc). About 90% of people with SSc have GI tract involvement, and understanding the connection between GI symptoms, their severity and abnormal GI transit may permit targeted therapeutic approaches for these patients.
Laura Hummers, MD, MSc, provided an update on the extra-pulmonary manifestations of scleroderma & the best ways to approach management of these issues.
ACR Convergence 2021—The past year has been an exciting time for researchers and clinicians focused on the topic of connective tissue disease-associated interstitial lung disease (CTD-ILD), with advances in our understanding of these disorders and newly approved medications for their treatment. At the ACR Convergence 2021 annual Review Course, Kristin Highland, MD, MSCR, director of…
Scleroderma renal crisis is a true medical emergency in rheumatology, one that requires prompt diagnosis and treatment. Here, we review the historic introduction of the angiotensin-converting enzyme inhibitors in this context, and highlight management and key questions moving forward. Background Awareness of renal disease in scleroderma dates back many years. The revered physician William Osler…
NEW YORK (Reuters Health)—Barrett’s esophagus is common in women with systemic sclerosis or scleroderma (SSc) and is often accompanied by dysplasia, according to the largest study on prevalence of Barrett’s esophagus in women with SSc. In Barrett’s esophagus, chronic gastric reflux causes the lining the esophagus to be replaced by metaplastic cells that may lead…
ACR CONVERGENCE 2020—In patients with connective tissue diseases, such as systemic sclerosis (SSc), pulmonary hypertension (PH) remains one of the leading causes of morbidity and mortality. In her lecture on Friday, Nov. 6, Pulmonary Hypertension: An Update, Mardi Gomberg-Maitland, MD, MSc, professor of medicine and medical director of the Pulmonary Hypertension Program at The George…
Patient with autoimmune disease may experience medical emergencies. Here is an overview of recognition and management of three emergent situations for patients with scleroderma: critical digital ischemia, scleroderma renal crisis and intestinal pseudo-obstruction…
Cancer and autoimmunity have a complex relationship. In a presentation, Ami Shah, MD, MHS, discussed how to use autoantibodies as tools for cancer risk stratification, how to approach cancer screening in individuals with new-onset disease and more…