A recently published study gives new insight into an innovative approach to treating patients with severe scleroderma and internal organ involvement. During the six-year study, researchers demonstrated the durability of the beneficial effects of stem cell transplantation in this patient population. Their data also suggest the treatment has the potential to renormalize the autoreactive immune system…
The year was 1991. It was my first Tuesday as a rheumatology fellow at the University of Pittsburgh’s Presbyterian Hospital. Navigating a maze of buildings and hallways, I delivered myself to the entrance to the scleroderma clinic. Running late and not knowing whether there was a separate entrance for staff, I clicked open the door….
SAN DIEGO—Fibrosis affects all organ systems, but isn’t always systemic sclerosis. Experts on less common forms discussed patient presentations, diagnosis and treatment at the 2017 ACR/ARHP Annual Meeting in San Diego on Nov. 6. Retroperitoneal Fibrosis Formerly called Ormond’s disease, retroperitoneal fibrosis (RPF) is usually an IgG4-related disease, but has some unique characteristics, said John…
During the ACR/ARHP Annual Meeting, Richard Pope, MD, highlighted some of the top scientific literature on rheumatic disease from 2017. The review session discussed research on the effects of microglia on the central nervous system of SLE patients, the role of adenosine in osteoarthritis and more…
Raynaud’s phenomenon in scleroderma or systemic sclerosis (SSc) is associated with significant discomfort and functional disability, especially in the presence of digital ulcers.1 Having lived with diffuse systemic sclerosis (dSSc) for nearly a decade, I can attest to this. It has been my experience that the hand pain and dysfunction in dSSc stems from Raynaud’s…
Systemic sclerosis (SSc) is a disease characterized by immunologic abnormalities, microvascular involvement and tissue fibrosis. In previous studies, 10-year survival rates ranged from 50–84%. However, there are concerns that these studies, using prevalent cohorts, are underestimating mortality. “While the prognosis of many rheumatic diseases has improved with the availability of more effective and targeted therapies,…
Systemic sclerosis (SSc), a subtype of scleroderma, is a rare, complex autoimmune disease characterized by widespread vasculopathy of the small arteries and fibroblast dysfunction.1,2 It has been described as a fibrosing microvascular disease, because vascular injury precedes and leads to tissue fibrosis.3 The resulting Raynaud’s phenomenon, pain, skin thickening and tightening, and multi-organ involvement have…
When it was created in 1982, the Division of Rheumatology and Immunology at the University of Nebraska Medical Center comprised one-and-a-half rheumatologists: its founder, Lynell W. Klassen, MD, MACR, and Gerald Moore, MD, who later received formal training at the NIH and now serves as senior associate dean for academic affairs. Thirty-five years later, the…
Systemic sclerosis (SSc) is a rare disease affecting about 49,000 U.S. adults, and it is strongly associated with high levels of morbidity and mortality.1 Of the few available antifibrotic therapies, none is targeted for SSc. However, reason for optimism exists for antifibrotic treatments in early development and clinical trials, says Jörg H.W. Distler, MD, Heisenberg Professor…
Rheumatologists now have another treatment option to offer their patients with one of, if not the most, difficult autoimmune diseases to treat. New data add to the growing evidence of the safety and benefit of stem cell transplantation for patients with systemic sclerosis with internal organ involvement. New results of the Scleroderma: Cyclophosphamide or Transplantation…