Updated systemic sclerosis criteria improve disease classification

October 3, 2013

New classification criteria for systemic sclerosis have just been published and are more sensitive than the 1980 criteria, enabling earlier identification and treatment of this disabling autoimmune disease. The 2013 criteria, developed by a joint committee commissioned by the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR), are published in the ACR journal, Arthritis & Rheumatism.

Systemic sclerosis, also known as scleroderma, is a connective tissue disease that is characterized by sclerodermatous skin changes–a hardening of tissue due to increased collagen deposits; Raynaud's phenomenon–spasms of small blood vessels in response to cold or stress that cause color changes in fingers or toes, to obliteration of blood vessels (vasculopathy) leading to tissue death; and internal organ fibrosis–formation of excess tissue that scars organs. The ACR estimates that affects 49,000 U.S. adults.

The joint ACR-EULAR committee was led by Dr. Janet Pope from Western University, St. Joseph's Health Care London in Ontario, Canada, and Dr. Frank van den Hoogen from St. Maartenskliniek in The Netherlands. The committee's intent was to improve the classification of systemic sclerosis by clustering items and simplifying the weighting of the different criteria. The new criteria set was tested for specificity and sensitivity by comparing scleroderma cases with controls (patients with disorders similar to scleroderma), and validated by experts viewing cases with and without the disease.

"There is a need for improved for systemic sclerosis," explains Dr. van den Hoogen. "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."

Based on the new criteria, a patient with thickening of the skin in the middle part of the fingers (from proximal to the metacarpophalangeal joints) would be classified as having systemic sclerosis, regardless of other features. If this criterion was not met, however, then seven items with varying weights would need to be assessed in order to obtain a scleroderma classification: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, pulmonary arterial hypertension and/or interstitial lung disease, Raynaud's phenomenon, and SSc-related antibodies.

The results of the validation testing show that sensitivity and specificity were both greater than 90% for the 2013 systemic sclerosis classification criteria compared to 75% for the 1980 ACR criteria. "The new systemic sclerosis classification criteria should correctly classify more patients with the disease," concludes Dr. Pope. "Criteria that are more specific will allow for earlier identification and better treatment for those with systemic sclerosis."

Explore further: Ultrasound findings can improve classification of RA

More information: "2013 Classification Criteria for Systemic Sclerosis. An American College of Rheumatology and European League Against Rheumatism Collaborative Initiative" Frank van den Hoogen, Dinesh Khanna, Jaap Fransen, Sindhu R. Johnson, Murray Baron, Alan Tyndall, Marco Matucci-Cerinic, Raymond P. Naden, Thomas A. Medsger Jr., Patricia E. Carreira, Gabriela Riemekasten, Philip J. Clements, Christopher P. Denton, Oliver Distler, Yannick Allanore, Daniel E. Furst, Armando Gabrielli, Maureen D. Mayes, Jacob M. van Laar, James R. Seibold, Laszlo Czirjak, Virginia D. Steen, Murat Inanc, Otylia Kowal-Bielecka, Ulf Mu¨ller-Ladner, Gabriele Valentini, Douglas J. Veale, Madelon C. Vonk, Ulrich A. Walker, Lorinda Chung, David H. Collier, Mary Ellen Csuka, Barry J. Fessler, Serena Guiducci, Ariane Herrick, Vivien M. Hsu, Sergio Jimenez, Bashar Kahaleh, Peter A. Merkel, Stanislav Sierakowski, Richard M. Silver, Robert W. Simms, John Varga and Janet E. Pope. Arthritis & Rheumatism; Published Online: October 3, 2013. DOI: 10.1002/art.38098

Related Stories

Ultrasound findings can improve classification of RA

May 15, 2013
(HealthDay)—Compared to clinical diagnosis of synovitis, ultrasound-detected synovitis provides either improved sensitivity or specificity when used with the American College of Rheumatology/European League Against Rheumatism ...

Study suggests systemic sclerosis is an independent risk factor for atherosclerosis

May 10, 2011
A new study by researchers in Hong Kong suggests that systemic sclerosis is an independent determinant for moderate to severe coronary calcification or atherosclerosis. Conventional cardiovascular risk factors such as age ...

Recommendations guide physicians in treatment of systemic juvenile arthritis

September 24, 2013
In the U.S., there are nearly 300,000 children with juvenile arthritis and other rheumatic illnesses according to estimates from the American College of Rheumatology (ACR). For pediatric patients with systemic juvenile idiopathic ...

Systemic sclerosis complications more severe in African Americans than Caucasians

May 10, 2012
African Americans have more severe complications from systemic sclerosis, also known as scleroderma, than Caucasians. Findings published today in, Arthritis & Rheumatism, a journal of the American College of Rheumatology ...

Haematopoietic stem cell transplantation increases survival in systemic sclerosis patients

June 7, 2012
Initial results from an international, investigator-initiated, open label phase III trial were presented at EULAR 2012, the Annual Congress of the European League Against Rheumatism. Data indicate that haematopoietic stem ...

Recommended for you

Fluid in the knee holds clues for why osteoarthritis is more common in females

June 26, 2017
Researchers have more evidence that males and females are different, this time in the fluid that helps protect the cartilage in their knee joints.

Biologics before triple therapy not cost effective for rheumatoid arthritis

May 29, 2017
Stepping up to biologic therapy when methotrexate monotherapy fails offers minimal incremental benefit over using a combination of drugs known as triple therapy, yet incurs large costs for treating rheumatoid arthritis (RA). ...

Drug for refractory psoriatic arthritis shows promise in clinical trial

May 24, 2017
In a pivotal phase-3 clinical trial led by a Stanford University School of Medicine investigator, patients with psoriatic arthritis for whom standard-of-care pharmaceutical treatments have provided no lasting relief experienced ...

Cross-species links identified for osteoarthritis

May 17, 2017
New research from the University of Liverpool, published today in the journal npj Systems Biology and Applications, has identified 'cell messages' that could help identify the early stages of osteoarthritis (OA).

Osteoarthritis could be prevented with good diet and exercise

May 12, 2017
Osteoarthritis can potentially be prevented with a good diet and regular exercise, a new expert review published in the Nature Reviews Rheumatology reports.

Rodents with trouble walking reveal potential treatment approach for most common joint disease

May 11, 2017
Maintaining the supply of a molecule that helps to nourish cartilage prevented osteoarthritis in animal models of the disease, according to a report published in Nature Communications online May 11.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.