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Progression Likely With Early Diffuse Cutaneous Systemic Sclerosis

Case fatality rate high despite early use of immunosuppressive therapies

By Physician’s Briefing Staff | November 01, 2021

Despite treatment, patients with early diffuse cutaneous systemic sclerosis (dcSSc) often die from organ damage, according to a study recently published in Arthritis Research & Therapy.

Sara Jaafar, M.D., from the University of Michigan in Ann Arbor, and colleagues longitudinally followed patients who had early dcSSc or who were at risk for dcSSc treated at one of 12 U.S. centers from April 2012 to July 2020. The analysis included 301 patients with no more than two years since the first non-Raynaud's phenomenon symptom with a median follow-up of 24.5 months.

The researchers reported that at baseline, 87.3 percent of patients had definite dcSSc and 12.7 percent were at risk. Overall, just under half of patients (49.6 percent) were positive for anti-RNA polymerase III antibodies. Nearly two-thirds of patients (63.1 percent) were treated with an immunosuppressive therapy, most commonly mycophenolate mofetil. Nearly three-quarters of the 38 patients who were classified as at-risk at baseline developed dcSSc. Those patients who progressed had higher baseline mean Health Assessment Questionnaire-Disability Index and higher baseline modified Rodnan skin scores versus those who remained as limited cutaneous SSc. Just over one in five patients (21.1 percent) had clinically significant worsening of skin fibrosis, mainly occurring in the first year of follow-up. Twenty participants (6.6 percent) died, mostly in the first three years of follow-up, with cardiac involvement (33.3 percent), gastrointestinal dysmotility (22.2 percent), and progressive interstitial lung disease (16.7 percent) as the main causes of death.

"Despite using the standard of care therapies, patients continue to have worse thickening of their skin and internal organ involvement, including lung scarring that has an impact on their disability, function and survival," a coauthor said in a statement.

Several authors disclosed ties to the pharmaceutical and biomedical industries.

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