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

Systemic Sclerosis

Hidradenitis Suppurativa Tied to Higher Mortality

David Douglas  |  

NEW YORK (Reuters Health)—Hidradenitis suppurativa (HS) is associated with a significantly increased risk of adverse cardiovascular (CV) outcomes and all-cause mortality, according to Danish researchers. HS is a chronic, inflammatory skin disease and is most common in women. It is typically diagnosed after long delays, Dr. Alexander Egeberg of the University of Copenhagen and colleagues…

Rheumatology Case Report: Chondromyxoid Fibroma of the Sternum

Rajaie Namas, MD, Reshma Khan, MD, & Bernard Rubin, DO  |  

A 47-year-old Caucasian woman presented to the rheumatology clinic with a one-year history of pain and swelling involving the wrists, hands, ankles and feet that progressively worsened. Her symptoms included generalized fatigue, morning stiffness in the hands and lower back lasting more than an hour, Raynaud’s phenomena (triphasic), photosensitivity, tightening of the skin of the…

New Evidence MRI Can Be Used to Monitor Neuromuscular Disease Progression

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In a study of patients with Charcot-Marie-Tooth disease, researchers found that MRI scans can be used to detect the muscle water changes that precede marked intramuscular fat accumulation that may contribute to the disease’s presentation. MRI biomarkers may prove useful in clinical trials for therapies for this and other neuromuscular disorders by enabling researchers to measure disease progression…

Fellow’s Forum Case Report: Limited Cutaneous Systemic Sclerosis

Eunjung Kim, MD, Hyun Bae, MD, Ritu Kathuria, MD, Alexandra Gottdiener, MD, & Girish Sonpal, MD, FACR  |  

Systemic sclerosis (SSc) is an acquired, sporadic, autoimmune, connective tissue disease with two subsets: limited cutaneous scleroderma (lcSSc) and diffuse cutaneous scleroderma (dcSSc). In the U.S., the annual incidence is about 20 cases per 1 million adults, with a prevalence of about 240 cases per 1 million adults.1 As with other connective tissue disorders, SSc…

The ACR’s State-of-the-Art Clinical Symposium: Stem Cell Therapy in Autoimmune Disease Evolution, Insights

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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,…

The ACR’s State-of-the-Art Clinical Symposium: Patients with Scleroderma, Lung Disease May Benefit from Aggressive Therapy

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CHICAGO—Scleroderma patients with pulmonary arterial hypertension (PAH) might benefit from more aggressive therapy, an expert in the field said in a session on lung involvement in rheumatic diseases at the American College of Rheumatology’s 2015 State-of-the-Art Clinical Symposium in May. Newer trials—the SERAPHIN trial on macitentan, GRIPHON on selexipag, and AMBITION on an ambrisentan/tadalafil combination—show…

Figures 1 & 2: On exam, the patient had thickening of the skin on her extremities.

Dermatology Symptoms Point to Connective Tissue Disorder

Natalie A. Wright, MD, and Joseph F. Merola, MD, MMSc, FAAD, FACR  |  

The Case A 68-year-old woman with a past medical history of Charcot-Marie-Tooth presents with thickening of the skin on her trunk and extremities, which she has had for the past seven months (see Figures 1 and 2). Her symptoms first began with swelling of her bilateral upper and lower extremities. She is now having difficulty…

Rehabilitation, Therapy Goals for Scleroderma, Acroosteolysis

Lauren Tarsi and Marie B. Corkery, PT, DPT, MHS, FAAOMPT  |  

Scleroderma is a rare rheumatologic auto­immune disease that affects the skin and can also affect other organs. Due to excess formation of scar tissue, blood flow to the extremities is decreased, primarily to the hands, and tissues often become hypoxic, resulting in sclerodactyly and proximal skin involvement.2 The incidence of scleroderma in the U.S. is…