CSRG patient medications (ever used unless otherwise stated).
| Therapeutic Agent or Investigation | Before June 2009, All SSc Patients, n = 992, Frequency (%) | After June 2009, All SSc Patients, n = 261, Frequency (%) | Comparing All Before and After June 2009, p |
|---|---|---|---|
| Raynaud’s phenomenon | 952 | 246 | — |
| Calcium channel blockers | 578 (60.9) | 139 (56.5) | 0.2091 |
| Iloprost | 16 (1.7) | 0 (0.0) | 0.0820 |
| PDE5 inhibitors | 17 (1.8) | 13 (5.3) | 0.0018 |
| Skin involvement in early diffuse SSc | |||
| Methotrexate use for diffuse SSc only, < 2 yrs’ duration | 21 (28.4) | 4 (25.0) | > 0.05 |
| Methotrexate use for diffuse SSc only, < 5 yrs’ duration | 48 (35.3) | 6 (22.2) | > 0.05 |
| Skin involvement (mRSS > 10) | 347 | 62 | — |
| Corticosteroids | 149 (43.1) | 20 (32.3) | 0.1117 |
| D-penicillamine | 74 (21.4) | 11 (17.7) | 0.5151 |
| Methotrexate | 105 (30.4) | 17 (27.4) | 0.6429 |
| Cyclophosphamide | 37 (10.7) | 6 (9.7) | 0.8103 |
| Skin involvement (mRSS > 20) | 141 | 25 | — |
| Corticosteroids | 63 (45.0) | 5 (20.0) | 0.0193 |
| D-penicillamine | 32 (22.9) | 2 (8.0) | 0.0907 |
| Methotrexate | 53 (37.9) | 3 (12.0) | 0.0119 |
| Cyclophosphamide | 16 (11.4) | 4 (16.0) | 0.7547 |
| Digital ulcers | 524 | 108 | — |
| Calcium channel blocker | 369 (70.7) | 77 (71.3) | 0.8996 |
| Iloprost | 15 (2.9) | 0 (0.0) | 0.1509 |
| Bosentan | 28 (5.4) | 2 (1.9) | 0.1187 |
| Digital ulcers (active; current use) | 86 | 13 | — |
| Calcium channel blocker | 67 (77.9) | 11 (84.6) | 0.8513 |
| Ilopost | 4 (4.7) | 0 (0.0) | 1.0000 |
| Bosentan | 7 (8.1) | 0 (0.0) | 0.5894 |
| Digital ulcers (healed) | 318 | 60 | — |
| Calcium channel blocker | 232 (73.2) | 44 (73.3) | 0.9812 |
| Bosentan | 18 (5.7) | 2 (3.3) | 0.6679 |
| GERD | 824 | 212 | — |
| Gastroprotective agents | 705 (86.2) | 174 (82.1) | 0.1316 |
| Promotility agents | 191 (23.4) | 49 (23.1) | 0.9422 |
| Delayed gastric emptying | 468 | 116 | — |
| Promotility agents | 194 (41.9) | 49 (42.2) | 0.9470 |
| Dysphagia | 194 | 64 | — |
| Promotility agents | 49 (25.3) | 20 (31.3) | 0.3477 |
| Dilatation | 29 (15.1) | 9 (14.5) | 0.9101 |
| Inflammatory arthritis | 92 | 32 | — |
| Methotrexate | 33 (36.3) | 10 (31.3) | 0.6089 |
| Hydroxychloroquine | 36 (39.6) | 9 (28.1) | 0.2480 |
| D-penicillamine | 17 (18.7) | 4 (12.5) | 0.4241 |
| NSAID | 53 (58.2) | 14 (43.8) | 0.1568 |
| Corticosteroids | 45 (49.5) | 15 (46.9) | 0.8020 |
| Inflammatory myositis/myopathy | 99 | 24 | — |
| Methotrexate | 39 (39.4) | 13 (54.2) | 0.1887 |
| Azathioprine | 15 (15.2) | 6 (25.0) | 0.3964 |
| Cyclophosphamide | 11 (11.1) | 2 (8.3) | 0.9784 |
| Renal crisis ever | 46 | 4 | — |
| Hemodialysis or peritoneal dialysis | 16 (34.8) | 2 (50.0) | 0.6123 |
| Kidney transplant | 5 (11.1) | 1 (25.0) | 0.4175 |
| ACE inhibitor | 39 (86.7) | 2 (50.0) | 0.1195 |
| ACE inhibitor (current use) | 29 (64.4) | 2 (50.0) | 0.6181 |
| PH by CSRG criteria† | 98 | 19 | — |
| Flolan | 8 (8.3) | 1 (5.3) | 1.0000 |
| Trepostinil | 3 (3.1) | 1 (5.3) | 0.5161 |
| Iloprost | 0 (0.0) | 0 (0.0) | — |
| Bosentan | 12 (12.4) | 0 (0.0) | 0.2273 |
| Sitaxsentan* | 2 (2.1) | 2 (10.5) | 0.1249 |
| PDE5 inhibitors | 2 (2.1) | 2 (10.5) | 0.1249 |
| Warfarin | 22 (22.7) | 2 (10.5) | 0.3755 |
| Combination PAH treatment | 13 (13.4) | 1 (5.3) | 0.5413 |
| PH† and NYHA ≥ Class III | 86 | 16 | — |
| Flolan | 7 (8.1) | 0 (0.0) | 0.5927 |
| Trepostinil | 4 (4.7) | 1 (6.3) | 0.5819 |
| Iloprost | 1 (1.2) | 0 (0.0) | 1.0000 |
| Bosentan | 15 (17.4) | 1 (6.3) | 0.4497 |
| Sitaxsentan* | 2 (2.3) | 2 (12.5) | 0.1150 |
| PDE5 inhibitors | 2 (2.3) | 2 (12.5) | 0.1150 |
| Warfarin | 22 (25.6) | 2 (12.5) | 0.4169 |
| Combination PAH treatment | 15 (17.4) | 2 (12.5) | 0.9031 |
| DLCO < 70% and TLC < 70% and FVC < 70% and short of breath | 106 | 34 | — |
| Corticosteroids | 58 (55.2) | 13 (38.2) | 0.0848 |
| Azathioprine | 6 (5.7) | 7 (20.6) | 0.0244 |
| Cyclophosphamide | 20 (19.1) | 3 (8.8) | 0.1632 |
| Mycophenolate mofetil | 2 (1.9) | 2 (5.9) | 0.2510 |
| ILD by CSRG criteria** | 347 | 68 | — |
| Corticosteroids | 149 (43.4) | 21 (30.9) | 0.0547 |
| Azathioprine | 37 (10.8) | 8 (11.8) | 0.8136 |
| Cyclophosphamide | 56 (16.3) | 7 (10.3) | 0.2072 |
| Mycophenolate mofetil | 10 (2.9) | 3 (4.4) | 0.7911 |
↵* Sitaxsentan has been removed from the market.
↵† CSRG definition of suspected pulmonary hypertension is pulmonary arterial pressure (PAP) measured using the Doppler flow measurement of the tricuspid regurgitation jet on echocardiography and an estimated systolic PAP > 45 mm Hg. For the subset analysis, only NYHA ≥ III were included.
↵** CSRG definition of ILD is a positive HRCT lung with ILD or abnormal chest radiograph with interstitial markings (not thought to be due to congestive heart failure) or fibrosis, and/or findings of ILD on physical examination. CSRG: Canadian Scleroderma Research Group; SSc: systemic sclerosis; mRSS: modified Rodnan skin score; PDE5: phosphodiesterase type 5; GERD: gastroesophageal reflux disease; NSAID: non-steroidal antiinflammatory drugs; ACE: angiotensin-converting enzyme; PH: pulmonary hypertension; NYHA: New York Heart Association; ILD: interstitial lung disease; HRCT: high-resolution computed tomography; TLC: total lung capacity; FVC: forced vital capacity.