Table 1.
Patient (N) | Age at diagnosis (years)/sex | Disease duration from diagnosis (years) | Type of SSc | Autoantibodies | Clinical involvement | Immunosuppressive drugs at sampling time | Other treatments | AOPP plasma level (chloramine-T equivalents, μmol/L) |
---|---|---|---|---|---|---|---|---|
1 | 54/F | 16 | d-SSc | Scl70 | CIPO, DU, GER, ILD, PAH | Low-dose steroids, azathioprine | Bosentana, tadalafila, low-dose steroids, cyclophosphamide, MMF, sildenafil, treprostinil, tadalafil, iloprost | 121 |
2 | 27/F | 16 | d-SSc | Scl70, ANA | DU, GER, ILD | None | Bosentana, sildenafila | 253 |
3 | 61/F | 2 | lc-SSc | Cm, PM/Scl | GER | None | None | 267 |
4 | 32/M | 25 | d-SSc | Scl70, PM/Scl, ANA | CIPO, DU, GER, ILD, PAH, My | MMF | D-penicillamine | 269 |
5 | 49/F | 14 | lc-SSc | ANA | GER, SRC | None | None | 293 |
6 | 21/F | 49 | lc-SSc | Scl70, ANA | DU, GER, ILD | None | None | 302 |
7 | 49/F | 10 | d-SSc | Scl 70 | J, CIPO, DU, GER, ILD, PAH, SC | Low-dose steroids | Bosentana, azathioprine, MTX, MMF, iloprost | 319 |
8 | 65/F | 0 | lc-SSc | Scl70, ANA | GER, ILD, PAH | None | None | 378 |
9 | 31/F | 27 | d-SSc | Scl70, SSa | DU, GER, ILD, PAH | MMF | Bosentana, sildenafila, iloprosta | 390 |
10 | 21/F | 1 | lc-SSc | ANA | DU, GER, ILD | None | Low-dose steroids | 394 |
11 | 22/F | 7 | lc-SSc | ANA | DU, GER, My | IvIg | None | 508 |
12 | 57/F | 16 | lc-SSc | Scl70, ANA | DU, GER, ILD | None | Bosentan, iloprost | 553 |
13 | 81/F | 3 | lc-SSc | ANA | My, PAH | Low-dose steroids, IvIg | None | 575 |
14 | 52/F | 17 | d-SSc | Scl70, ANA | J, DU, GER, ILD, SC | None | Bosentan | 613 |
15 | 46/F | 29 | lc-SSc | Cm, ANA, CCP | GER, SC | Low-dose steroids, MTX | Leflunomide, abatacept | 677 |
16 | 47/F | 8 | d-SSc | ANA | J, DU, GER, SC, SRC | None | None | 886 |
17 | 49/F | 6 | lc-SSc | Cm, PM/Scl | DU, GER, SC | None | Bosentana | 1,248 |
All patients presented with Raynaud phenomenon and were treated with symptomatic treatments (calcium channel blockers, proton pump inhibitors).
ANA, antinuclear antibody; AOPP, advanced oxidation protein products; CCP, anticyclic citrullinated peptide; CIPO, chronic intestinal pseudo-obstruction; Cm, anticentromere antibody; d-SSc, diffuse systemic sclerosis; DU, digital ulcers; F, female; GER, gastroesophageal reflux; ILD, interstitial lung disease; IvIg, intravenous immunoglobulins; J, joints; lc-SSc, limited cutaneous systemic sclerosis; M, male; MMF, mycophenolate mofetil; MTX, methotrexate; My, myositis; PAH, pulmonary arterial hypertension; Scl70, anti-Scl70 antibody; SC, subcutaneous calcinosis; SRC, scleroderma renal crisis; SSa, anti-Ro/SSa antibody; SSc, systemic sclerosis.
aOther treatment at sampling time.