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Mod Rheumatol. 2019 Nov;29(6):977-983. doi: 10.1080/14397595.2018.1551265. Epub 2019 Jan 3.

Less severe disease in patients with early systemic sclerosis?

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Rheumatology Division, Universidade Federal do Mato Grosso do Sul, Mato Grosso do Sul, Brazil.
Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil.


Objectives: To evaluate the disease severity and activity in patients with a diagnosis of systemic sclerosis (SSc) after the 2013 American College of Rheumatology and the European League Against Rheumatism (ACR/EULAR) classification criteria development compared to patients diagnosed before 2013.Methods: One hundred and fifty-four subjects were included and assigned to the following groups: 120 SSc patients meeting the 1980 ACR criteria and with a diagnosis before 2013 (historical group), and 34 patients diagnosed after 2013, fulfilling the new ACR/EULAR criteria (early SSc group). Disease activity was assessed by the 2001 European Scleroderma Study Group Activity Index (EScSG-AI) and by the revised European Scleroderma Trials and Research group (EUSTAR) activity index. Disease severity was assessed using the Medsger Disease Severity Scale (DSS) and the summed DSS score.Results: The time between the first non-Raynaud's symptom and the diagnosis was shorter in early SSc than in the historical group (p = .001). The EScSG-AI and the EUSTAR activity index were similar between groups. The summed DSS score and the general, skin and gastrointestinal tract DSS scores were significantly lower in early SSc than in the historical group.Conclusion: SSc patients with a diagnosis after the new ACR/EULAR criteria development were diagnosed earlier and had a less severe disease than historical patients.


Disease activity; disease severity; scleroderma; systemic sclerosis

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