Abstract
ObjectivesTo evaluate the performance of the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in clinical practice in a Chinese patient cohort, and to compare outcomes with the 1980 ACR criteria.MethodsPatients clinically diagnosed with SSc between September 2013 and May 2015 were prospectively recruited from the EUSTAR database of the Peking Union Medical College Hospital. Diagnosis of SSc was based on the evaluation of three experienced rheumatologists. Patients diagnosed with other connective tissue diseases were recruited as disease controls. The 1980 ACR and 2013 ACR/EULAR criteria were applied to the cohort, and patients who fulfilled the criteria were classified as definite SSc patients. Sensitivity and specificity were analyzed for the 2013 and 1980 criteria.ResultsA total of 143 SSc patients and 87 patients with other connective diseases were recruited. 41 (28.7%) and 102 (71.3%) cases were diffuse cutaneous SSc and limited cutaneous SSc, respectively. Although the sensitivity of the 2013 criteria (94.4%) exceeded the 1980 criteria (72.7%) (P<0.001), the 1980 and 2013 criteria sets showed no significant difference in specificity (97.7% and 93.1%, respectively, P = 0.278). The sensitivity of the 2013 criteria was significantly higher than the 1980 criteria in some SSc subgroups (e.g., lcSSc, abnormal pattern of nailfold videocapillaroscopy [NVC] and presence of Raynaud’s phenomenon [RP]) compared to others.ConclusionsRelative to the 1980 ACR criteria, in Chinese SSc patients the new 2013 ACR/EULAR criteria had similar specificity and higher sensitivity, especially for patients with mild skin thickening or prominent microvascular diseases.
Highlights
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis, microvascular disease, and presence of autoantibodies
The sensitivity of the 2013 criteria was significantly higher than the 1980 criteria in some SSc subgroups
A joint committee of the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) formulated a new set of SSc classification criteria, which has proven to have increased sensitivity over the 1980 criteria while still preserving the specificity determined from studies conducted in other countries [5,6]
Summary
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis, microvascular disease, and presence of autoantibodies. The first widely accepted classification criteria for SSc were published in 1980, and the high specificity of 1980 criteria helped minimize false-positive diagnoses [3]. Improved classification criteria that could diagnose SSc even earlier were urgently needed. In 2001 LeRoy and Medsger proposed modifications to the criteria used to diagnose early SSc [4]. A joint committee of the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) formulated a new set of SSc classification criteria, which has proven to have increased sensitivity over the 1980 criteria while still preserving the specificity determined from studies conducted in other countries [5,6]. The objective of this study was to validate the sensitivity and specificity of the new classification criteria in Chinese SSc patients
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