Estimated risk of death for each group compared with the general Japanese population.
| Group | Standardized Mortality Ratio (95% CI) |
|---|---|
| All patients | 2.76 (2.18–3.35)* |
| Subgroup | |
| Male | 3.31 (1.15–5.47)* |
| Female | 2.71 (2.10–3.32)* |
| dcSSc | 5.90 (4.20–7.61)* |
| lcSSc | 1.71 (1.18–2.24)* |
| Onset < 45 yrs | 3.99 (2.71–5.28)* |
| Onset ≥ 45 yrs | 2.24 (1.61–2.87)* |
| Anti Scl-70 antibody (+) | 4.57 (2.82–6.33)* |
| ACA (+) | 0.86 (0.35–1.37) |
| Anti U1-RNP antibody (+) | 2.40 (1.14–3.65)* |
| GI involvement (+) | 3.43 (2.47–4.39)* |
| Lung involvement (+) | 5.04 (3.90–6.18)* |
| Heart involvement (+) | 8.01 (5.62–10.40)* |
| Kidney involvement (+) | 4.74 (2.88–6.60)* |
| PAH (+) | 5.67 (3.53–7.81)* |
| SLE (+) | 2.63 (–0.35–5.61) |
| RA (+) | 4.68 (0.09–9.26) |
| SS (+) | 0.81 (–0.11–1.73) |
| Myositis (+) | 10.09 (3.10–17.07)* |
| Death from | |
| Malignancy | 1.70 (0.94–2.47) |
| Pneumonia (interstitial or infectious) | 9.11 (4.34–13.88)* |
↵* SMR significantly > 1. SMR: standardized mortality ratio; dcSSc: diffuse cutaneous SSc; lcSSc: limited cutaneous SSc; ACA: anticentromere antibodies; GI: gastrointestinal; PAH: pulmonary arterial hypertension; SLE: systemic lupus erythematosis; RA: rheumatoid arthritis; SS: Sjögren’s syndrome.