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…
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…
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….
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…