Table 3.

Summary of frequency of organ complications in systemic sclerosis (SSc).

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.