CREST Syndrome in Systemic Sclerosis Patients – Is Dystrophic Calcinosis a Key Element to a Positive Diagnosis?

Figures & data

Table 1 The New ACR/EULAR Classification Criteria from 2013 for SSc Diagnosis

Diagnostic Criteria	Score
Skin sclerosis/thickening of the fingers proximal to the metacarpophalangeal joints	9
Sclerodactyly or digital edema	4/3
Fingertip pulp ulcers or stellate scars	2/3
Telangiectasia	2
Abnormal capillaries	2
Lung fibrosis	2
Lung arterial hypertension	2
SSc specific antinuclear antibodies  - anti-centromere  - anti-Scl-70  - anti-RNA polymerase III	3
Raynaud’s phenomena	2

Note: Data from Masi AT, Medsger TA Jr.²⁵

Table 2 Frequency Distribution by SSc ACR Criteria, in Both Subgroups and Total Group of Patients

SSc ACR Criteria		Limited SSc		Diffuse SSc		Total		Chi-Square	p
		n	%	n	%	n	%
1 major criterion	No
	Yes	14	100.0%	23	100.0%	37	100.0%
2 minor criteria	No	8	57.1%	4	17.4%	12	32.4%	4.590	0.032
	Yes	6	42.9%	19	82.6%	25	67.6%
Total		14	100.0%	23	100.0%	37	100.0%

Note: Bold value indicates statistically significant.

Figure 1 Clinical elements of CREST syndrome: Frequency distributions for the total group and by subsets of SSc.

Table 3 CREST Syndrome Elements – Patient Group and Disease Subgroups Frequency Distribution

CREST Syndrome Elements		Limited SSc		Diffuse SSc		Total		Chi-Square	p
		n	%	n	%	n	%
Calcinosis	No	10	71.4	15	65.2	25	67.6	0.153	0.493, NS.
	Yes	4	28.6	8	34.8	12	32.4
Raynaud phenomena	No	1	7.1			1	2.7	1.688	0.378,NS
	Yes	13	92.9	23	100.0	36	97.3
Esophageal dysfunction	No	7	50.0	7	30.4	14	37.8	1.416	0.234,NS
	Yes	7	50.0	16	69.6	23	62.2
Sclerodactyly	No	4	28.6	3	13.0	7	18.9	1.368	0.228,NS
	Yes	10	71.4	20	87.0	30	81.1
Telagiectasia	No	6	42.9	6	26.1	12	32.4	1.117	0.242,SS
	Yes	8	57.1	17	73.9	25	67.6
Total		14	100.0%	23	100.0%	37	100.0%

Abbreviations: NS, not statistically significant; SS, statistically significant.

Masi AT, Medsger TA Jr. Progress in the evolution of systemic sclerosis classification criteria and recommendation for additional comparative specificity studies. J Rheumatol. 2015;42(1):8–10. doi:10.3899/jrheum.141020

 PubMed Web of Science ®Google Scholar