Background/Purpose: To assess the newly established ACR/EULAR classification criteria for systemic sclerosis ¹ (SSc) in a large Norwegian SSc registry cohort.

Methods: The Norwegian systemic connective tissue disease and vasculitis registry (NOSVAR) at the Department of Rheumatology, Oslo University Hospital (OUH) was interrogated for all systemic sclerosis (SSc) patients. All the SSc patients included in NOSVAR fulfil either the American Rheumatism Association 1980 classification criteria for SSc (ACR criteria) and/or LeRoy and Medsger’s modified criteria for the classification of early SSc. Patients are scored according to the algorithm in the new ACR/EULAR criteria and classified as SSc if scoring 9 points or more. Descriptive statistics are applied to analyse the number of patients meeting the newly established classification criteria for SSc.

Results: NOSVAR includes 381 patients with SSc. 270 SSc patients (71%) fulfil the ACR criteria. The remaining 111 SSc patients meet the modified Medsger and LeRoy criteria for SSc. The mean age at onset is 48 years (SD 0.8), the median disease duration is 10 years (range 1-48 years) and the female to male ratio is estimated to 4:1. The cohort consists of 7 limited (l) SSc patients, 277 limited cutanoeus (lc) SSc patients and 98 diffuse cutaneous (dc) SSc patients. Altogether, 73/381 patients (19%) have bilateral skin thickening extending proximal to the metacarpophalangeal (MCP) joints and are classified as SSc by this criterion alone. Of the remaining 302 patients, 296 gain >9 points by fulfilling other parameters defined in the new ACR/EULAR criteria. Only 6 patients (2%) in the NOSVAR cohort do not meet the new criteria. These 6 patients fulfil only the LeRoy and Medsger’s modified SSc criteria and have Raynauds phenomenon, pathological capillaroscopi and a positive anti-nuclear antibody, but no skin changes. 

Conclusion: This study demonstrates the applicability of the new SSc classification criteria in a large, clinical registry cohort. Only 2% of SSc patients in the cohort do not meet the new ACR/EULAR criteria.

 References

1. Van den Hoogen, F et al. Classification criteria for Systemic Sclerosis; preliminary results. Annals Rheum Dis 2013;72 (3): 59.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/assessment-of-the-new-american-and-european-classification-criteria-for-systemic-sclerosis-in-the-norwegian-systemic-connective-tissue-disease-and-vasculitis-registry/