Items assigned points in the scoring system are:
- Skin thickening of the fingers;
- Fingertip lesions;
- Telangiectasia;
- Abnormal nailfold capillaries;
- Pulmonary arterial hypertension and/or interstitial lung disease;
- Raynaud’s phenomenon; and
- Scleroderma-related autoantibodies (antitopoisomerase I, anticentromere, anti–RNA polymerase III).
The scoring system (see Table 1), kept to a maximum of 19 possible points with weights confined to single digits, should simplify its use, particularly given that similar systems are used for other rheumatic diseases, such as rheumatoid arthritis. Dr. Pope anticipates the creation of application software that will make the scoring system easier to use when classifying patients with SSc.
According to Dr. Pope, clinicians may wonder why certain items were not included in the list of classification criteria. Many were excluded because they were considered redundant, she says. For example, scleroderma renal crisis was excluded. “It is very specific but rare, so patients are usually classified by other features,” she says.
Some gastrointestinal features, such as lower esophageal dysphagia, dilated esophagus, and gastric antral vascular extasia, which are common in SSc and somewhat specific, were also considered redundant, “as other features are usually present to include these patients in the classification,” she says.
Cost of potential treatment and effect of the new criteria on insurance coverage were not part of the ACR/EULAR decision making or research in drafting this document. Dr. Pope noted that more patients potentially will be correctly classified with SSc and get access to medications that are indicated for certain organ system involvement. “There will be more overlap in the new criteria with clinical judgment where, previously, some patients were labeled with SSc but did not meet the former criteria.”
Kathy Holliman is a medical journalist based in New Jersey.
References
- Ringold S, Weiss PF, Beukelman T, et al. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: Recommendations for the medical treatment of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologic medications. Arthritis Care Res. 2013;65: 1551-1563.
- van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: An American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65:2737-2747.