What are the ACR/EULAR Revised Systemic Sclerosis Classification Criteria?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

In 2013, a joint committee of the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) published a revised classification criteria for systemic sclerosis [1, 2] to improve the sensitivity of the widely used previous classification criteria. The revised criteria for the classification of systemic sclerosis are listed in Table I, below.

Table. (Open Table in a new window)

Table 1: ACR/EULAR Revised Systemic Sclerosis Classification Criteria

Item

Sub-item(s)

Score*

Skin thickening of the fingers of both hands extending proximally to the metacarpophalangeal joints (presence of this criterion is sufficient criterion for SSc classification)

None

9

Skin thickening of the fingers (count the higher score only)

Puffy fingers

2

Sclerodactyly (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints)

4

Fingertip lesions (count the higher score only)

Digital tip ulcers

2

Fingertip pitting scars

3

Telangiectasia

None

2

Abnormal nailfold capillaries

None

2

Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)

Pulmonary arterial hypertension

2

Interstitial lung disease

2

Raynaud phenomenon

None

3

Systemic sclerosis–related autoantibodies (maximum score is 3)

Anticentromere

3

Anti–topoisomerase I

3

Anti–RNA polymerase III

3

*The total score is determined by adding the maximum score in each category. Patients with a total score equal to or greater than 9 are classified as having definite systemic sclerosis (modified from 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. Nov 2013;65(11):2737-47. [1] )

Historical background

Hippocrates first described this condition as thickened skin. [3] In 1836, Giovambattista Fantonetti used for the first time the term “skleroderma generale” to describe a patient with dark leather-like skin who exhibited a loss of range of joint motion caused by skin tightening. [4] Robert H. Goetz introduced the concept of scleroderma as a systemic disease in 1945 and coined the term progressive systemic sclerosis to emphasize the systemic and often progressive nature of the disease. [5]

The purpose of classification criteria is to enroll a uniform population of patients in research studies and it is based on various parameters that are commonly used for systemic sclerosis diagnosis. It is important to emphasize that these criteria are not diagnostic criteria and are not applicable to patients with scleroderma-like disorders or to patients with skin thickening sparing the fingers.


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