Severe Organ Involvement | No.* | Frequency, % | 95% CI |
---|---|---|---|
1. Scleroderma renal crisis (SRC) | |||
1.1 SRC in dcSSc | 2,848 | 12 | 5–19 |
1.2 SRC in SSc | 14,482 | 3 | 3–4 |
2. Severe cardiac involvement | |||
2.1 Cardiomyopathy in SSc† | 7,700 | 3 | 1–6 |
2.2 Clinical congestive heart failure in SSc† | 1,431 | 5 | 1–9 |
2.3 Congestive heart failure or symptomatic pericarditis/symptomatic arrhythmia/cardiomyopathy in dcSSc† | 1,178 | 13 | 10–17 |
2.4 Congestive heart failure or symptomatic pericarditis/symptomatic arrhythmia/cardiomyopathy in SSc† | 1,024 | 15 | 6–24 |
2.5 Diastolic dysfunction in SSc† | 10,484 | 16 | 14–17 |
2.6 Symptomatic conduction system abnormality or arrhythmia attributable to scleroderma heart disease requiring treatment in SSc | 11,140 | 17 | 14–21 |
2.7 Symptomatic pericarditis or moderate-large pericardial effusion in SSc† | 1,302 | 3 | 1–5 |
3. Pulmonary hypertension (PH) & pulmonary artery hypertension (PAH) | |||
3.1 PH in SSc (echo) | 6,571 | 14 | 8–20 |
3.2 PH in SSc (sPAP > 35 mm Hg)† | 2,242 | 14 | 9–19 |
3.3 PH in SSc (sPAP > 40 or 45 mm Hg) | 11,533 | 18 | 14–21 |
3.4 PH in SSc (sPAP > 50 or 55 mm Hg)† | 691 | 19 | 5–32 |
3.5 PAH in SSc (right heart catheterization) | 4,292 | 15 | 12–17 |
3.6 PH with pulmonary fibrosis in SSc† | 5,021 | 11 | 4–19 |
4. Pulmonary restriction in SSc | |||
4.1 Forced vital capacity % predicted < 80%† | 965 | 17 | 12–21 |
4.2 Forced vital capacity % predicted < 70%† | 2,669 | 15 | 12–17 |
4.3 Forced vital capacity % predicted < 50%† | 1,875 | 8 | 4–13 |
4.4 Total lung capacity % predicted < 80%† | 2,102 | 23 | 22–25 |
5. Malabsorption, intestinal pseudoobstruction, hyperalimentation in SSc† | 1,788 | 5 | 3–6 |
6. Severe musculoskeletal involvement | |||
6.1 Proximal muscle weakness in SSc† | 9,670 | 24 | 17–30 |
6.2 Myopathy or myositis in dcSSc† | 359 | 9 | 0–17 |
6.3 Myositis in SSc | 4,134 | 13 | 10–17 |
7. Articular involvement | |||
7.1 Clinical arthritis in SSc | 11,890 | 12 | 9–16 |
7.2 Radiographic change in those with clinical arthritis in SSc† | 774 | 9 | 4–14 |
8. Digital ulcer and complications | |||
8.1 Prevalent digital ulcer in SSc | 5,261 | 15 | 10–20 |
8.2 Current digital gangrene in SSc† | 1,557 | 2 | 1–3 |
8.3 Digital amputation in SSc† | 1,756 | 12 | 8–16 |
8.4 Hospitalization due to digital vascular ischemic complication or overall digital vascular ischemic complications in SSc† | 3,248 | 13 | 6–21 |
9. Overlap Sjögren’s syndrome in SSc† | 1,903 | 13 | 10–16 |
↵* Number of patients from included studies;
↵† overall estimated prevalence that metaanalysis was conducted for ≤ 4 studies. dcSSc: diffuse cutaneous systemic sclerosis; lcSSc: limited cutaneous systemic sclerosis; PH: pulmonary hypertension; PAH: pulmonary artery hypertension; sPAP: systolic pulmonary artery pressure.