Background/Purpose:  Localized scleroderma (LS) is characterized by skin sclerosis, producing devastating impairment in those affected. It was previously thought to “burn out” within 5 years, but recent studies have challenged this notion. However, no studies have prospectively examined LS disease activity over time. Understanding the course of LS is necessary to determine how to counsel and evaluate patients and to plan outcome and interventional studies. The aim of the present study was to determine the disease course of LS using a validated clinical outcome measures in a prospective cohort of patients.

Methods:  Prospective cohort study of 131 participants from the Morphea in Adults and Children (MAC) cohort with at least two years of follow-up and modified LS skin severity scores (mLoSSI) scores recorded. Study visits were conducted at six to twelve month intervals. Disease activity was defined as mLoSSI score greater than 0. Time to recurrence of disease activity from the first visit with inactive disease was compared between the linear and generalized subtype using survival analysis with the log-rank (Mantel-Cox) test. All statistical analysis was performed using GraphPad Prism 6.0.

Results:  131 participants (670 study visits) were included. Fifty had at least 5 years of follow-up. Mean total follow-up was 4.3±1.7 years. The majority of participants were Caucasian (72%), female (77%), and had either a linear (55%) or generalized (31%) subtype. Median baseline mLoSSI score was 5. The mean time to first recurrence of disease activity after initial resolution of activity was 1.1 years for generalized LS and 2.3 years for linear LS. Overall, 44% of those with generalized LS had a recurrence of disease activity compared to 21% of those with the linear subtype (Hazard ratio 2.79, 95% CI 1.48-7.94). Of the 50 participants with at least five years of follow-up, 56% had a recurrence of disease activity. All but three subjects had similar or milder disease activity than the initial presentation.

Conclusion:  Disease activity improves in the majority of LS patients over time. Some patients with LS have a monophasic disease course in which activity resolves. However, a substantial number of patients have a relapsing remitting course over many years. Specifically, those with the generalized subtype appear to be at a higher risk for future disease flares. Patients with LS warrant monitoring for disease flares for extended periods of time.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/determining-disease-course-in-localized-scleroderma-a-prospective-cohort-study/