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Table 1 Patient demographics

From: Linaclotide for the treatment of refractory lower bowel manifestations of systemic sclerosis

Clinical variable

n = 31

Age at first linaclotide dose, mean (SD)

52 (11)

Female sex, % (n)

94 (29)

White race, % (n)

68 (21)

SSc type: Diffuse cutaneous disease, % (n)

32 (10)

Disease duration in years, RP or non-RP, median (IQR)

9 (5–14)

Medsger GI score at initiation of linaclotide, % (n)

 

 0 (Normal)

0 (0)

 1 (Requiring medications for reflux or abnormal small bowel series)

6 (2)

 2 (High-dose reflux medications or antibiotics for bacterial overgrowth)

19 (6)

 3 (Malabsorption syndrome or episodes of pseudo-obstruction)

65 (20)

 4 (Requiring total parental nutrition)

10 (3)

Sicca symptoms, % (n)

84 (26)

Myopathy, % (n)

42 (13)

Cardiac involvement (Medsger ≥ 1), % (n)

55 (17)

Lung involvement (Medsger > 1), % (n)

71 (22)

Raynaud’s (Medsger ≥ 2), % (n)

61 (19)

Antibody status

 

 Anti-Scl-70 antibodies, % (n)

23 (7)

 Anti-CENP antibodies, % (n)

23 (7)

 Anti-RNA pol-3 antibodies, % (n)

10 (3)

 Anti-U3RNP antibodies, % (n)

13 (4)

UCLA GIT 2.0 constipation score, mean (SD) n = 9

1.03 (0.55)

Whole gut scintigraphy data

 

 Abnormal large bowel transit at 72 h, % (n)

70 (7/10)

 Percent large bowel emptying at 72 h, median (IQR)

9.5 (0–84)

Medications used prior to linaclotide

 

 Polyethylene glycol, % (n)

71 (22)

 Senna, % (n)

32 (10)

 Oral docusate, % (n)

42 (13)

 Docusate suppository, % (n)

13 (4)

 Tegaserod, % (n)

10 (3)

 Lubiprostone, % (n)

29 (9)

 Pyridostigmine, % (n)

13 (4)

 Prucalopride, % (n)

3 (1)

  1. UCLA GIT (None-to-mild: 0.00–0.49); Whole gut scintigraphy (Normal % colonic emptying at 72 h ≥ 67%)