Table 1.
Clinical, serological characteristics, organ involvement, and therapies at baseline of the 21/25 patients with systemic sclerosis.
| Characteristics | |
|---|---|
| Disease subtype and clinical manifestations | |
| lcSSc | 17/21 (81) |
| dcSSc | 4/21 (19) |
| Years from RP (median, range) | 14 (1–50) |
| RP | 20/21 (95.2) |
| Scleroderma | 1/21 (4.8) |
| Median modified Rodnan skin score (median, range) | 1.5 (0–10) |
| Telangiectasia | 18/21 (85.7) |
| History of and/or active DU and/or pitting scars | 13/21 (61.9) |
| Sinovytis | 1/21 (4.8) |
| Muscle weakness and/or CK elevation | 9/21 (42.8) |
| Melanoderma | 1/21 (4.8) |
| Gastrointestinal involvement | 17/21 (81) |
| History of renal crisis | 0 |
| Autoantibodies | |
| ANA | 21/21 (100) |
| ACA | 9/21 (42.9) |
| ATA | 10/21 (47.6) |
| RNA pol III | 0 |
| PM-Scl | 0 |
| Fibrillarin | 0 |
| Negative SSc-marker autoantibodies | 0 |
| Features of lung involvement | |
| FVC < 80% predicted | 7/21 (33.3) |
| DLCO < 80% predicted | 16/21 (76.2) |
| ILD on HRCT of the lungs | 10/21 (47.6) |
| EScSG-AI (median, range) | 1.75 (0–3) |
| EScSG-AI ≥ 3 | 3/21 (14.3) |
| Therapies | |
| Proton pump inhibitors | 21/21 (100) |
| Low-dose aspirin | 21/21 (100) |
| Calcium channel blockers | 21/21 (100) |
| Glucocorticoids + vitamin D | 16/21 (76) |
| Immunosuppressants | 11/21 (52) |
| ACE-I/ARB | 12/21 (57) |
| Prostanoids | 1/21 (5) |
Values are expressed as n (%), except where otherwise indicated.
ACA, anticentromere antibodies; ACE-I, angiotensin-converting enzyme inhibitors; ANA, antinuclear antibodies; ARB, angiotensin receptor blockers; ATA, anti-topoisomerase I antibodies; dcSSc, diffuse cutaneous systemic sclerosis; DLCO, diffusing lung capacity for carbon monoxide expressed as a percentage of the predicted value; DU, digital ulcers; EScSG-AI, European Scleroderma Study Group-Activity Index; FVC, forced vital capacity expressed as a percentage of the predicted value; HRCT, high resolution computed tomography; ILD, interstitial lung disease; lcSSc, limited cutaneous systemic sclerosis; RNA pol III, anti-RNA polymerase III; RP, Raynaud’s phenomenon; SD, standard deviation; SSc, systemic sclerosis.