Background/Purpose: Systemic sclerosis (SS), is characterized by fibrosis of the skin and internal organs and vasculopathy with relatively high morbidity and mortality.  Given the rarity of the disease, studying epidemiology, treatment and natural history is challenging without large patient cohorts. We conducted a cross-sectional study to understand the prevalence, disease characteristics, demographics, and treatment patterns ofSS patients using the Rheumatology Informatics System for Effectiveness (RISE) EHR-based registry.

Methods:

Between 2015 and 2017, we identified patients who had at least one physician diagnosis code of SS (M34.x or M34.xx) and aged 18 years or older at the first encounter. The demographics included age, gender, race, region, and body mass index (BMI). We identified relevant comorbidities that associated with scleroderma including other autoimmune diseases, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). In addition we evaluated relevant medications including prednisone, immunomodulation, calcium channel blockers (CCB), phosphodiesterase type 5 (PDE 5) inhibitors, endothelin receptor antagonists (ERA), proton pump inhibitors (PPIs), H2 blockers, angiotension-converting enzyme inhibitors (ACEIs), angiotensin II receptor antagonist (ARBs) and opioids. We identified comorbidities associated with scleroderma using ICD 9/10 diagnosis codes, and identified medications using prescriptions or associated procedures. We also evaluated the patients’ characteristics across different practice sites.

Results:

We identified 12,057 scleroderma patients who had >=1 physician diagnosis in RISE 2015-2017, representing 210 separate sites. After limiting to patients who were 18 year or older, 10,286 patients were eligible for the analysis. The majority of SS patients were female (88.5%) and white (56.8), with average age of 59.6 (SD: 13.8) years. 11% and 12% of patients had a diagnosis codes for ILD and PAH, respectively. Among all eligible patients, 24.5% ever had prednisone, 42% had immunomodulation, 33% had CCB, 9.1% had PDE5, 3.2% have ERAs, 42.7% had PPIs, 10.7% had ACEI, 8.2% had ARBs, and 16.1% had opioids. Only 25 sites reported orders for pulmonary function testing (PFTs) with the proportion of patients undergoing these ranging between 4 and 90% per site.

Conclusion: The RISE registry serves as one of the largest patient cohorts for systemic sclerosis. It is comprised largely of patients from community practices, identified by rheumatologists. The demographics and identified treatments may differ from other academic cohorts but potentially better represents management of patients seen in a general practice. PFT use varies significantly amongst practices, however this finding may be more a reflection of how the registry captures this data and where these tests are performed which is a limitation of this cohort. Disclaimer: This data was supported by the ACR’s RISE Registry. However, the views expressed represent those of the authors, not necessarily those of the ACR.

« Back to 2019 ACR/ARP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-of-patients-with-systemic-sclerosis-in-the-rheumatology-informatics-system-for-effectiveness-rise-registry/