New Scleroderma Criteria Improves Upon Disease Classification

The American College of Rheumatology (ACR), together with the European League Against Rheumatism (EULAR) published new criteria for classification of systemic sclerosis. The new guidelines were introduced to allow earlier diagnosis and facilitate more targeted therapeutic treatments. The criteria were published in the journal of Arthritis & Rheumatism in a report entitled “2013 Classification Criteria for Systemic Sclerosis. An American College of Rheumatology and European League Against Rheumatism Collaborative Initiative.”

Scleroderma, also known as systemic sclerosis, is an autoimmune disease characterized by skin thickening, a process known as fibrosis. While in some types this hardening is confined to the skin on the head, face and feet, in more severe cases, it affects internal organs such as the kidneys, heart, lungs, and intestine. The disease is currently without an approved treatment and is associated with high mortality rates, with 49,000 U.S. adults estimated to be affected by the disease, according to the ACR.

The new classification system, developed by the ACR-EULAR committee, was led by Dr. Janet Pope from Western University in Canada and Dr. Frank van den Hoogen from St. Maartenskliniek in The Netherlands. The commission introduced new criteria that are more specific and sensitive, and were established by evaluating scleroderma cases and comparing them to patients with other disorders similar to scleroderma.

Dr. van den Hoogen noted, “There is a need for improved classification criteria for systemic sclerosis. The 1980 ACR criteria were not sensitive enough to identify patients with early disease or limited cutaneous system sclerosis. Our efforts with the joint committee addressed this sensitivity issue, resulting in the 2013 classification criteria for systemic sclerosis.”

According to the new criteria, a patient exhibiting skin thickening in the fingers’ metacarpophalangeal joint is diagnosed with systemic sclerosis, independently of other symptoms. In the absence of this condition, other features need to be determined for a systemic sclerosis diagnosis. These include skin thickening of the fingers, fingertip lesions, telangiectasia (small dilated blood vessels near the surface of the skin), abnormal nailfold capillaries, pulmonary arterial hypertension and/or interstitial lung disease, Raynaud’s phenomenon, and systemic sclerosis-related antibodies.

The new criteria established levels of specificity and sensitivity above 90% for systemic sclerosis classification criteria. Dr. Pope added, “The new systemic sclerosis classification criteria should correctly classify more patients with the disease. Criteria that are more specific will allow for earlier identification and better treatment for those with systemic sclerosis.”