Research Article

Endothelin-1 Levels in Scleroderma Patients: A Pilot Study

Table 1

Correlation between clinical subsets (DfSSC, LcSSc, CREST, and overlap syndrome), presence of ulcers, serological data (ANA, anti-ENA, and ET-1 concentrations), and different treatments (CA, bosentan).

PatientDUsTherapyAnticentromere Anti-ENAET-1 concentration (pg/mL)
1° serum 2° serum1° serum 2° serum1° serum 2° serum

(1) DfSSc, PAHYesBosentanNeg.Neg.Scl 70 (127,2)Scl 70 (130,8)8,16,9
(2) DfSScNoCANeg.Neg.Scl 70 (46,9);
SS-A (143)
Scl 70 (75,0);
SS-A (143,8)
7,33,7
(3) LcSSc,YesCANeg.Neg.Neg.Neg.4,64,6
(4) LcSSc, PAHNoBosentanPos.Pos.Neg.Neg.5,74,6
(5) LcSSc, PAHYesBosentanPos.Pos.Neg.Neg.4,83,7
(6) LcSScNoCANeg.Neg.Scl 70 (108,8)Scl 70 (136,6)4,64,4
(7) LcSSc, PAHNoBosentanPos.Pos.Neg.Neg.3,83,5
(8) LcSScYesCANeg.Neg.SS-A (132,1)SS-A (133,5)244,1
(9) LcSSc, PAHNoBosentanPos.Pos.Neg.Neg.6,04,9
(10) LcSScYesCAPos.Pos.Neg.Neg.4,354,6
(11) LcSScNoCAPos.Pos.SS-A (33,0)SS-A (43,8)5,04,6
(12) LcSScYesCAPos.Pos.Neg.Neg.4,24,2
(13) LcSScYesCAPos.Pos.Neg.Neg.4,03,6
(14) LcSScNoCANeg.Neg.Neg.Neg.4,03,7
(15) LcSScNoCAPos.Pos.Neg.Neg.4,33,75
(16) LcSScNoCANeg.Neg.Scl 70 (133,4);
SS-A (41,4)
Scl 70 (134,0);
SS-A (44,6)
3,34,5
(17) CRESTNoCAPos.Pos.Neg.Neg.4,33,2
(18) DfSScNoCANeg.Neg.Scl 70 (102,3);
SS-A (128,1);
SS-B (127,1)
Scl 70 (121,0);
SS-A (133,8);
SS-B (131,0)
6,47,2

DfSSc: diffuse scleroderma; LcSSc: limited scleroderma; PAH: pulmonary arterial hypertension; DUs: digital ulcers; ANA: anti-nuclear antibodies; anticentromere: anticentromere antibodies; anti-ENA (Scl 70, SS-A, SS-B): anti-extractable nuclear antigen antibodies; ET-1: endothelin-1; Neg.: negative; Pos.: positive.