1980 Systemic Sclerosis (Scleroderma) Classification Criteria

These criteria were established in 1980 by the American Rheumatism Association (now known as the American College of Rheumatology).

Requirements

  • Either the sole major criterion or ≥2 of the minor criteria.

Major Criterion

  • Proximal scleroderma: symmetrical thickening, tightening and induration of the skin of the fingers and the skin proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes can involve the entire limb, face, neck and trunk.

Minor Criteria

  • Sclerodactyly: induration and tightening of the skin of the fingers.
  • Digital ischemia: as manifested by digital pitting scars or atrophy of finger pads.
  • Bibasilar pulmonary fibrosis: reticular or reticulonodular densities most pronounced in the basilar areas of the lungs on CXR. This may produce the appearance of “honeycomb lung” and must not be due to a primary pulmonary disease.

Deficiencies

  • Fails to include some patients with limited scleroderma or CREST Syndrome.
  • Does not include subtle features of the disease.
  • Does not include serological markers.

Adapted from: Masi AT, Rodnan GP, Medsger et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23:581-590.

2013 ACR/EULAR Classification Criteria for Scleroderma

These criteria were developed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR).  Twenty-three candidate items were considered, and reduced by clustering items and simplifying weights. The system was tested by comparing performance in scleroderma patients and control patients and validated with a group of experts.

Skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for a patient to be classified as having scleroderma. If this is not present, seven other additive items are considered, with varying weights for each (see below).

Patients with a total score of ≥ 9 are classified as having definite scleroderma.