TABLE 2

Risk factors for mortality and disease progression in systemic sclerosis-associated interstitial lung disease (SSc-ILD)

First author [ref.]Study design and patient numbersIndependent risk factor(s)Measure of progression
Steen [73]Analysis of 890 patients evaluated in a US centre between 1972 and 1989Disease severity (FVC % pred)10-year cumulative survival
Tyndall [4]Analysis of data from 2940 patients in the EUSTAR databaseFVC <80% pred and DLCO <80% predMortality
Zhang [74]Analysis of 1043 patients from the Canadian Scleroderma Research Group (multicentre database)Symptoms of oesophageal dysmotilityLow FVC (<70%)
Ahmed [75]Observational cohort of 188 patients form the Toronto Scleroderma ProgrammeBaseline 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 yearsHigher age at onset, dcSSc, lower FVC and DLCO, presence of anti-topoisomerase I antibodiesClinically 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 clinicBaseline FVC, 6-min walk distance1-year mortality
Okamoto [77]Retrospective analysis of 35 patients with SSc-ILDUsual interstitial pattern on HRCT, higher score for ground-glass attenuation with traction bronchiectasis on HRCTMortality
Elhai [5]Analysis of data from 11 193 patients in the EUSTAR databaseILD, DLCO <60% pred, FVC <70% predMortality
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 mRSSMortality
Becker [79]Analysis of 706 patients with diffuse SSc and 12 months of follow-up from the EUSTAR databaseAdvanced age (>60 years), active digital ulcer; lung fibrosis (FVC <60% or FVC <70% with presence of fibrosis on HRCT), muscle weakness, elevated C-reactive proteinDisease progression#
Hoffmann-Vold [2]Prospective Norwegian cohort study of 815 patients with SSc>25% fibrosis on HRCTMortality
Hoffmann-Vold [30]Analysis of 826 patients with FVC measures available at baseline and after 12 months from the EUSTAR databaseMale sex, higher mRSS, presence of gastro-oesophageal reflux disease at baselineFVC 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.