Background/Purpose:

Standard treatment for the diverse aspects of diffuse cutaneous systemic sclerosis (dcSSc) is not yet well defined although experts have described therapeutic algorithms.  The Prospective Registry of Early Systemic Sclerosis (PRESS) is longitudinal cohort of patients with early dcSSc at 11 US centers enrolling since 2012. In this study we describe the medical treatments utilized by PRESS investigators throughout the duration of the study and their changes over time.

Methods:

Adult patients with a diagnosis of dcSSc and early disease as defined as <2 years since the onset of the first non-RaynaudÕs symptom are included. Associations of treatment choices with demographic factors and organ involvement were assessed using logistic regression and FisherÕs exact test.

Results:

As of May 2018, baseline data were available on 239 patients with characteristics listed in table 1. Follow-up data was available on 168 (70.3%.) The median follow-up time was 12 months.

During the study period, 68.4% were noted to have Interstitial lung disease (ILD). At baseline, 60.3% of patients were on an immunosuppressive agent, the majority of whom were taking MMF (37.2%.) During follow-up, MMF was the most commonly prescribed immunosuppressive and was taken by 151 patients (63.2%) followed by MTX by 50 (20.9%), and HCQ by 39 (16.3%). Seventy-four (31.1%) of patients used prednisone at baseline but its use waned over time. Forty (16.7%) patients remained off immunosuppressive therapy throughout the course of follow-up. The use of these drugs over time is shown in table 2.  

Immunosuppressive choice was noted to be associated with specific demographic factors. Patients disabled due to scleroderma at the baseline visit were less likely to have previously received MMF, odds ratio (OR) 0.27 (0.07 – 0.96), p = 0.044, and patients with RA overlap were more likely to have received MMF, OR 3.26 (1.06 – 10.06), p= 0.040. Use of MMF at baseline was not associated with more severe Modified Rodnan Skin Score (MRSS). MTX use at baseline was more common in patients with higher MRSS scores, but this trend lost significance when looked at over the first year. Looking at medication use through one year of follow up, patients were more likely to be prescribed MMF if they had ILD defined at baseline (p=0.043) and less likely to receive MTX (p=0.034). Prednisone use was more common in those patients with overlapping RA or polymyositis.

Conclusion:

Most patients enrolled in the PRESS registry are treated with immunosuppressive agents, most commonly MMF or MTX. Treatment choice varies with disease manifestations and severity.