Risk factors for mortality and disease progression in systemic sclerosis-associated interstitial lung disease (SSc-ILD)
| First author [ref.] | Study design and patient numbers | Independent risk factor(s) | Measure of progression |
| Steen [73] | Analysis of 890 patients evaluated in a US centre between 1972 and 1989 | Disease severity (FVC % pred) | 10-year cumulative survival |
| Tyndall [4] | Analysis of data from 2940 patients in the EUSTAR database | FVC <80% pred and DLCO <80% pred | Mortality |
| Zhang [74] | Analysis of 1043 patients from the Canadian Scleroderma Research Group (multicentre database) | Symptoms of oesophageal dysmotility | Low FVC (<70%) |
| Ahmed [75] | Observational cohort of 188 patients form the Toronto Scleroderma Programme | Baseline FVC pred <70% and DLCO pred <77%, higher age at baseline (adjusted for FVC and DLCO) | Mortality |
| Nihtyanova [71] | Single-centre cohort of 398 consecutive patients with SSc followed for up to 15 years | Higher age at onset, dcSSc, lower FVC and DLCO, presence of anti-topoisomerase I antibodies | Clinically significant pulmonary fibrosis (FVC or DLCO <55% pred or documented decline in FVC or DLCO <15%) |
| Ryerson [76] | Application of four risk-prediction models (derived from IPF) to 156 patients recruited from a specialised SSc-ILD clinic | Baseline FVC, 6-min walk distance | 1-year mortality |
| Okamoto [77] | Retrospective analysis of 35 patients with SSc-ILD | Usual interstitial pattern on HRCT, higher score for ground-glass attenuation with traction bronchiectasis on HRCT | Mortality |
| Elhai [5] | Analysis of data from 11 193 patients in the EUSTAR database | ILD, DLCO <60% pred, FVC <70% pred | Mortality |
| Volkmann [78] | Long-term, follow-up analysis of patients in SLS I and II (up to 12 years in SLS I (median 8 years), n=158; up to 8 years in SLS II (median 4 years), n=142) | Decline in FVC and DLCO over 24 months, increased age, increased mRSS | Mortality |
| Becker [79] | Analysis of 706 patients with diffuse SSc and 12 months of follow-up from the EUSTAR database | Advanced age (>60 years), active digital ulcer; lung fibrosis (FVC <60% or FVC <70% with presence of fibrosis on HRCT), muscle weakness, elevated C-reactive protein | Disease progression# |
| Hoffmann-Vold [2] | Prospective Norwegian cohort study of 815 patients with SSc | >25% fibrosis on HRCT | Mortality |
| Hoffmann-Vold [30] | Analysis of 826 patients with FVC measures available at baseline and after 12 months from the EUSTAR database | Male sex, higher mRSS, presence of gastro-oesophageal reflux disease at baseline | FVC decline over a 5-year period |
FVC: forced vital capacity; EUSTAR: European Scleroderma Trials and Research; DLCO: diffusing capacity of the lungs for carbon monoxide; dcSSc: diffuse cutaneous SSc; IPF: idiopathic pulmonary fibrosis; HRCT: high-resolution computed tomography; SLS: Scleroderma Lung Study; mRSS: modified Rodnan skin score. #: new renal crisis, decrease of lung or heart function, new echocardiography-suspected pulmonary hypertension or death.