LeRoy and Medsger proposed criteria for the limited form of systemic sclerosis. The authors are from the Medical University of South Carolina and the University of Pittsburgh.
Patient selection: presence of scleroderma of the skin
Parameters
(1) subjective evidence for Raynaud's phenomenon
(2) objective evidence for Raynaud's phenomenon
(3) nailfold capillaroscopy (microscopic examination of capillaries in the nailfold)
(4) serologic evidence of autoantibodies (systemic sclerosis selective autoantibodies)
Subjective evidence of Raynaud's phenomenon on cooling - 2 or more of the following:
(1) pallor (well-demarcated whitening of acral skin)
(2) cyanosis (dusky blueness that disappears on re-warming)
(3) suffusion (well-demarcated erythema)
Objective evidence of Raynaud's phenomenon on cooling - one or both of the following:
(1) objective evidence of delayed recovery after cold challenge
(2) abnormal finger systolic pressure (DSP) during cooling (Nielsen test)
Abnormal widefield nailfold capillaroscopy - one or both of the following:
(1) abnormal dilatation and/or telangiectasia
(2) avascular areas
Autoantibodies may include one or more of the following in a titer >= 1:100:
(1) anti-centromere
(2) anti-topoisomerase I
(3) anti-fibrillarin
(4) anti-PM-Scl
(5) anti-fibrillin
(6) anti-RNA polymerase I
(7) anti-RNA-polymerase III
Criteria for the diagnosis of limited systemic sclerosis - one of the following:
(1) objective evidence of Raynaud's phenomenon AND abnormal nailfold capillarsocpy
(2) objective evidence of Raynaud's phenomenon AND serologic evidence of autoantibodies
(3) subjective evidence of Raynaud's phenomenon AND abnormal nailfold capillarscopy AND serologic evidence of autoantibodies
A limited form of systemic sclerosis may overlap with any other disease.
