Background/Purpose: Systemic Sclerosis (SSc) is a rare auto-immune connective tissue disease with an increasing burden for health care systems. National SSc registries have proven to be useful internationally to capture and track patient information in chronic diseases, such an example is the irish SSc registry running from 2011. The objective of our study was to characterize disease trends in the Irish SSc cohort.
Methods: A standard proforma was devised to capture relevant data fields for both the Irish Registry and European Scleroderma Trials and Research Group Registry (EUSTAR). Physical and functional assessments were carried out and captured. SSc cases in the Irish and EUSTAR database entered from March 2011-June 2012 were analysed in the following subsets (n= 67): limited (lcSSc), diffuse (dcSSc), and overlap/undifferentiated (Other) disease in comparison with each other.
Results: Mean±SD patient age was 57.41±13.22 years with disease onset of 13.95±10.19 years. LcSSc patients were older than dcSSc and Other counterparts (p=0.021). Beside the expected female predominance in each group, there were more male patients in the dcSSc vs any other groups (p=0.018). The onset of Raynaud’s Phenomenon was the shortest in dcSsc with 9.6+7.4 yrs (lcSSc: 15+10.8, Other: 15+10.4) and other SSc related symptoms also presented earlier in this subset with 6.13+5.9 yrs (lcSSc: 9.5+9.1, Other: 9.28+7.5 yrs). Gastrointestinal involvement was similar in each group (66.7%, 62%, 55%). DcSSc group was more frequently affected by cardiovascular (40% vs 25.8% vs 15%), lung (42.9% vs 16.7% vs 36.4 %) and musculoskeletal involvement (60% vs 20% vs 50%, p=0.0015) than lcSSc and Other subsets. The dcSSc group also manifested higher modified Rodnan skin scores (mRSS) than their counterparts (p<0.001). Comparing dcSSc with lcSSc based on a background of lung fibrosis, pulmonary arterial hypertension (PAH) was greater in the diffuse group (13.3% vs 9.7%, p=0.0015) as well as digital ulcer occurrence (13.3% vs 6.5%). LcSSc showed less frequent capillaroscopic scleroderma pattern (70% vs 100%) and aScl70 positivity (10.3% vs 58.3%, p<0.0001), however ACA positivity was higher compared to dcSSc (57.1 vs 25.0 %, p=0.002).
Conclusion: This is the first snapshot of disease characteristics of systemic sclerosis patients participating in the Irish SSc registry and EUSTAR. The continued enrolment of all SSc patients onto a national registry will facilitate the improvement in disease classification of the different subsets in the Irish population. A specialist nurse dedicated to SSc patients has been vital for information gathering to facilitate future research into this complex, challenging disease.
Disclosure:
M. Murray,
AbbVie, Pfizer, MSD, Roche,
2;
E. Balogh,
AbbVie, Pfizer, MSD, Roche,
2;
D. J. Veale,
AbbVie, Pfizer, MSD, Roche,
2,
Pfizer, Roche,
5,
Abbott, Pfizer, MSD, Roche,
8.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-development-of-a-national-systemic-sclerosis-service-in-ireland-incorporating-a-national-registry/