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Table 1 Patients’ characteristics, listed for all patients, and inflammatory, non-inflammatory, and intermediate systemic sclerosis (SSc)

From: Inflammatory stays inflammatory: a subgroup of systemic sclerosis characterized by high morbidity and inflammatory resistance to cyclophosphamide

 All patients (n = 131)Inflammatory (n = 24)Non-inflammatory (n = 38)Intermediate (n = 69)p value3
Age [years]54 (18–78)60 (24–77)50 (20–78)55 (18–77) 0.041
Male (n/%)24/18.39/384/1111/16°0.022
Body mass index [kg/m2]24.3 (16.4–38.1)25.4 (16.4–36.8)23.0 (18.8–33.4)25.6 (19.3–38.1)#0.069
Disease duration [years]0.0 (0–27.8)0.4 (0.0–22.1)0.0 (0.0–10.2)0.3 (0.0–27.8)#0.053
Number of visits per patient13 (3–38)13 (5–26)11 (3–21)16 (3–38)#0.365
Observation time [years]7.6 (1.0–19.5)5.8 (1.0–16.1)7.0 (1.9–15.8)9.3 (1.1–19.5)#°0.245
dcSSc/lcSSc (%)42/5867/3337/6336°/64°0.036
Scl-70 (n/%)53/4016/6712/3225/36°0.009
Anti-centromere (n/%)59/454/1723/6132/46°0.001
Anemia (n/%)28/218/337/1813/190.229
mRSS10 (0–41)18 (0–41)7 (0–22)10 (0–31)°< 0.001
FVC (n = 101) [%]92 (33–132)76 (33–108)102 (64–132)92 (62–130)°< 0.001
TLC (n = 100) [%]85 (44–118)76 (44–118)92 (58–117)86 (54–117)°0.001
DLCO (n = 96) [%]75 (26–121)57 (28–101)83 (39–121)77 (26–101)0.008
Pulmonary fibrosis (n/%)60/4620/8311/2929/42°< 0.001
Calcinosis cutis (n = 130/%)26/206/258/2112/180.762
Arthritis (n/%)26/206/258/2112/170.762
Rheumatoid arthritis (n = 130/%)10/85/210/05/70.007
Pulmonary hypertension1 (n/%)25/196/257/1812/170.541
Cardiac arrhythmia (n = 130/%)18/146/252/510/140.048
Arterial hypertension (n/%)55/4213/5413/3429/420.186
Atherosclerosis (n/%)16/124/171/311/160.069
Coronary artery disease (n = 130/%)11/83/130/08/12#0.056
Congestive heart failure (n/%)3/22/80/01/10.146
Esophageal involvement (n/%)90/6917/7125/6648/700.784
Gastrointestinal involvement (n = 128/%)19/152/83/814/211.000
Sjögren’s Syndrome (n = 127/%)8/60/02/56/90.515
Malignancy (n = 128/%)23/186/258/219/140.762
Prednisone (n/%)51/3915/639/2427/390.003
Immunosuppressive treatment ever* (n/%)72/5519/7914/3739/570.002
Methotrexate2 (n/%)33/258/334/1121/300.046
Azathioprine2 (n/%)19/153/133/813/190.669
Leflunomide2 (n/%)9/73/132/54/60.366
Cyclosporine2 (n/%)5/41/42/52/31.000
Mycophenolate mofetil2 (n/%)38/2910/428/2120/290.094
Abatacept2 (n/%)2/21/40/01/10.387
Rituximab2 (n/%)15/115/212/58/120.098
Cyclophosphamide2 (n/%)30/2312/505/1313/19°0.007
  1. Values are shown as median (range) or absolute numbers/percentages (n = 131 if not specified)
  2. CRP C-reactive protein, dcSSc diffuse cutaneous SSc, DLCO diffusing capacity, FVC forced vital capacity, lcSSc limited cutaneous SSc, mRSS modified Rodnan skin score, TLC total lung capacity
  3. *Immunosuppressive treatment including methotrexate, azathioprine, leflunomide, cyclosporine, mycophenolate mofetil, rituximab, abatacept, and cyclophosphamide
  4. #p value ≤ 0.05 as determined by Mann-Whitney U test or chi-square test comparing intermediate and non-inflammatory patients
  5. °p value ≤ 0.05 as determined by Mann-Whitney U test or chi-square test comparing intermediate and inflammatory patients
  6. 1Confirmed by right heart catheterization
  7. 2Number of patients who have ever received the listed treatment
  8. 3p values were determined by Mann-Whitney U test or chi-square test comparing inflammatory and non-inflammatory patients