Demographics of the study population
| Patient no/sex/age | Disease duration, months | Organ involvement at baseline* | Past immune-based therapy | Concomitant medication | SSc-specific anti-nuclear antibodies† | ||||
| Lung‡ | Renal§ | Heart¶ | Musculoskeletal** | GI†† | |||||
| 1/F/55 | 9 | + | + | + | + | + | d-penicillamine, oral corticosteroids, PUVA, etanercept, cyclophosphamide and methotrexate | Methotrexate 15 mg IM/week and guanethedine | Anti-RNA-polymerase III |
| 2/F/57 | 30 | + | − | − | + | − | Salazopyrine, oral corticosteroids and methotrexate | Methotrexate 15 mg IM/week and prednisolone 10 mg/day | Anti-topoisomerase I |
| 3/M/69 | 11 | − | + | − | + | − | − | − | Anti-RNA-polymerase III |
| 4/M/50 | 34 | − | − | + | + | − | Methotrexate and oral corticosteroids | Methotrexate 15 mg IM/week and methyl-prednisolone 8 mg/day | Anti-topoisomerase I |
| 5/M/49 | 22 | + | − | + | + | − | Methotrexate and oral corticosteroids | Methotrexate 25 mg IM/week and methyl-prednisolone 8 mg/day | Anti-topoisomerase I |
| 6/F/54 | 9 | + | − | − | + | − | Oral corticosteroids | Prednisolone 7.5 mg/day | Anti-topoisomerase I and anti-centromeric protein B |
| 7/M/51 | 9 | − | − | + | + | − | Methotrexate and oral corticosteroids | Methotrexate 15 mg IM/week and prednisolone 10 mg/day | Anti-RNA-polymerase III |
| 8/M/53 | 8 | + | − | + | + | − | − | − | − |
*All patients had truncal skin involvement. †All patients were positive for antinuclear antibodies (indirect immunofluorescence on HEp-2000 cells). ‡Lung involvement was defined as alveolitis or fibrosis on high resolution computed tomography (patient 1), restrictive lung function test (total lung capacity ⩽80%) (patients 2 and 5) or both (patients 6 and 8). §Renal involvement was defined as antecedent scleroderma renal crisis (patient 3), proteinuria (⩾0.3 g/l) or estimated glomerular filtration rate less than 60 ml/minute per 1.73 m2 (stage 3 of chronic kidney disease) (patients 1 and 3). ¶Cardiac involvement was defined as conduction disturbances (patients 7 and 8), left ventricular ejection fraction less than 55%, systolic pulmonary artery pressure greater than 40 mm Hg, pericardial effusion or diastolic dysfunction (patient 1, 3, 4, 5 and 8). **Musculoskeletal involvement was defined as joint contractures (patients 1, 2, 3, 4, 5, 6, 7 and 8), synovitis, muscle weakness (patients 1 and 4), or creatine kinase elevation. ††Gastrointestinal (GI) involvement was only technically investigated when new clinical complaints were present, and defined as gastrointestinal motility disturbance by barium swallow (patient 1), malabsorption, oesophageal stenosis, gastro-oesophageal reflux or intestinal pseudo-obstruction. Patients 2, 4, 5 and 8 were already on proton pump inhibitors before screening and had no further investigations. IM, intramuscularly; PUVA, psoralen–ultraviolet A.