Table 1.
Demographics, clinical characteristics and medications of study subjects
| Patient no/sex/age in years | Disease Duration in years | Type of Disease | Organ Involvement | Concurrent medications |
|---|---|---|---|---|
| 1/F/47 | 5 | limited | GI | - |
| 2/F/62 | 12 | diffuse | Lung,PAH,GI | CYC |
| 3/M/55 | 2 | limited | Lung,GI | - |
| 4/F/53 | 12 | diffuse | Lung,DU,GI | MMF, Bos |
| 5/F/53 | 12 | limited | Lung,DU,GI | Pred, RTX, Bos |
| 6/F/78 | 4 | diffuse | Lung,DU,PAH,Musc | Pred, Bos, RTX |
| 7/F/57 | 21 | limited | Lung,DU,GI | Pred, Bos |
| 8/F/58 | 3 | diffuse | Lung,GI | RTX |
| 9/F/66 | 9 | limited | Lung,GI | MMF |
| 10/F/59 | 17 | limited | Musc | – |
| 11/F/65 | 8 | limited | Lung,GI | RTX |
| 12/F/40 | 22 | diffuse | Lung,GI,Musc | RTX |
| 13/F/81 | 15 | limited | GI | – |
Pred: low dose prednisone; Bos: bosentan; MMF: mycophenolate mofetil; CYC: cyclophosphamide; RTX: rituximab
DU: digital ulcers; Musc: musculoskeletal involvement; GI: gastrointestinal involvement; PAH: pulmonary arterial hypertension