Systemic Sclerosis

An Update

Uwe-Frithjof Haustein, MD, PhD

Disclosures

Lab Med. 2011;42(9):562-572. 

In This Article

Definition and Criteria

The American College of Rheumatology (formerly American Rheumatism Association [ARA]) has defined criteria that are 97% sensitive and 98% specific for SSc as follows[1]

Major criterion:

  • Proximal diffuse (truncal) sclerosis (skin tightness, thickening, non-pitting induration)

  • Minor criteria:

  • Sclerodactyly (only fingers and/or toes)

  • Digital pitting scars or loss of substance of the digital finger pads (pulp loss)

  • Bibasilar pulmonary fibrosis.

  • The patient should fulfill the major criterion or 2 of the 3 minor criteria.

Raynaud's phenomenon is observed in 90%-98% of SSc patients.[2] Raynaud's phenomenon is a vasospastic disorder causing discoloration of the fingers and toes. It includes cyclic color changes: 1) starting with pale or white color (pallor) of the skin, which becomes cold and numb due to vasoconstriction followed by 2) blue color (cyanosis) due to depleted oxygen supply and turning into 3) red (rubor) due to reactive hyperemia. In extreme cases (eg, in SSc), Raynaud's phenomenon can progress to necrosis or gangrene of the fingertips (rat bite necrosis). Raynaud's phenomenon may precede SSc for years, and its presence may have a predictive value for the subsequent development of SSc, in particular in association with abnormal nailfold capillaries and the occurrence of antinuclear antibodies (ANA).[2,3]

As a result, the American College of Rheumatology criteria from 1980 is in need of possible revision, particularly to more adequately incorporate patients with limited SSc who do not meet the criteria established in 1980. Therefore, simple clinical variables, such as nail capillary microscopy and anti-centromere antibody (ACA) positivity, should be added as novel minor criteria. With these 2 new criteria, the sensitivity of ARA preliminary criteria was improved from 33% to 97%.[4,5]

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