Despite the innovations of new biologics and disease-modifying anti-rheumatic drugs, a large unmet need remains for patients with rheumatic autoimmune disease. Treatment remains limited for many conditions, including for conditions with a dim prognosis, such as systemic sclerosis.1 One promising treatment avenue is hematopoietic stem-cell transplantation (HSCT). Here, we provide background on HSCT for severe…
Search results for: diffuse systemic sclerosis
Ethics Forum: Physicians Face Ethical Quandary Discussing Poor Prognosis with Patients
Over the course of a month, you diagnose systemic sclerosis in two newly evaluated patients. Their responses to the news could not be more different. Patient 1 is a previously healthy 55-year-old man who is an avid bicyclist and skier. He presents with a several-month history of rapidly progressive skin tightening extending to the proximal…
The ACR/ARHP Award Winners Discuss Their Contributions to Rheumatology
At the 2015 ACR/ARHP Annual Meeting in San Francisco in November, the ACR and the ARHP honored a group of distinguished individuals who have made significant contributions to rheumatology research, education and patient care. In the December 2015 issue, The Rheumatologist reported on the ACR’s awards. This month, we speak with the ARHP winners about…
The ACR’s State-of-the-Art Clinical Symposium: Stem Cell Therapy in Autoimmune Disease Evolution, Insights
CHICAGO—Stem cell transplantation for systemic sclerosis patients has come a long way over the past decade, with more finely calibrated dosing and better patient selection, said George Georges, MD, associate member of the Fred Hutchinson Cancer Research Center and associate professor in the medical oncology division at the University of Washington School of Medicine, Seattle,…
Ethics Forum: The Ethical Side of End-of-Life Decisions
The role of the consulting physicians in the ICU, the concept of medical futility, and the management of complex patients when the goals of care may be changing.
Clinical Year in Review: 2023
SAN DIEGO—At ACR Convergence 2023, Philip Seo, MD, MHS, presented an overview of clinical updates in rheumatology from the past year.
Case Report: Pneumatosis Cystoides Intestinalis
Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal pathological process defined by the presence of gas within the layers of the intestinal wall, commonly within the mucosa and submucosa of the small and large intestines.2,3 PCI has been described in the literature in association with various connective tissue diseases, including scleroderma, mixed connective tissue disease,…
Case Report: Hajdu-Cheney Syndrome
Hajdu-Cheney syndrome (HCS) is a rare connective tissue disease, with fewer than 100 cases reported worldwide.1 Hallmark features include acro-osteolysis (i.e., resorption of the distal phalanges of the hands and feet), osteoporosis, facial dysmorphisms, and craniofacial and dental abnormalities. Patients often have short stature and can have neuroanatomical deformities causing intellectual disabilities. These patients can…
ACR Image Competition 2022 Results, Part 6
Diffuse Cutaneous Scleroderma with Interstitial Lung Disease: Radiological Findings A 44-year-old woman with cutaneous manifestations, tightening of her face with a reduced oral aperture and a healing digital tip ulcer (Figures 1 and 2) has had a known case of diffuse scleroderma since 2008. The patient presented with complaints of shortness of breath along with…
ACR Image Competition 2022 Results, Part 5
Resolution of GAVE After aHSCT for Progressive Systemic Sclerosis A 30-year-old man with RNA polymerase III (Pol III)-positive, diffuse, progressive systemic sclerosis had a persistent microcytic anemia with a hemoglobin level of 8 g/dL and evidence of gastric antral ventral ectasia (GAVE) on gastroscopy. He underwent autologous hematopoietic stem cell transplantation (aHSCT). After six months,…
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