2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative

Abstract

Background The 1980 classification criteria for systemic sclerosis (SSc) lack sensitivity in early SSc and limited cutaneous SSc. A joint ACR-EULAR committee was established to develop new classification criteria for SSc. Methods Using consensus methods, 23 candidate items were arranged in a multi-criteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items, and simplifying weights. The system was tested by: a) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders; b) validating against the combined view of a group of experts on a set of cases with or without SSc. Results Skin thickening of the fingers extending proximal to the MCPs is sufficient to be classified as SSc, if that is not present, seven additive items apply with varying weights for each: skin thickening of the fingers, finger tip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ARA classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc by the 1980 ARA criteria were classified with the new criteria, and several additional cases were now considered to be SSc. Conclusion The ACR-EULAR classification criteria for SSc performed better than the 1980 ARA Criteria for SSc and should allow for more patients to be classified correctly as SSc.

Molecular Basis for Dysregulated Activation of NKX2‐5 in the Vascular Remodeling of Systemic Sclerosis

“…All patients had a definite diagnosis of SSc, and the majority fulfilled the American College of Rheumatology/European League Against Rheumatism 2013 criteria for the classification of SSc 18. Patients were categorized according to disease subsets (limited/diffuse) or the presence of the following autoantibodies: anticentromere, antitopoisomerase, and anti–RNA polymerase III.…”

Personality and Psychopathology in Patients with Systemic Sclerosis

“…We included RA and SSc in-patients undergoing their routine medical check-up in the clinic. Diseases were diagnosed according to the American College of Rheumatology (ACR) criteria [16,17]. All of the enrolled patients met the following criteria: (1) RA or SSc diagnosis; (2) lack of overlap syndrome; (3) age ≥ 18 years old; and (4) intact cognitive functioning evaluated with the Mini-Mental State Examination [18].…”

Diagnosis and treatment of interstitial lung disease in scleroderma systematica

“…Клинико-иммунологические ассоциации имеют важное значение для установления клинической формы ССД на раннем этапе (диффузная или лимитированная), прогнозирования развития органной патологии (ИПЛ или ЛАГ), определения темпов прогрессирования и прогноза [7]. Важность выявления «склеродермических» аАТ для диагно-стики ССД подчеркивает то, что они включены в новые классификационные критерии заболевания [8].…”