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Table 1 Study population characteristics (n = 15)

From: A prospective, open-label, non-comparative study of ambrisentan with anti-fibrotic agent combination therapy in the treatment of diffuse systemic sclerosis

Age, mean (SD)

47.6 (10.7)

Race, n (%)

 White

14 (93)

 Black or African American

1 (7)

Sex, n (%)

 female

10 (67)

 male

5 (33)

Presence of ANA, n (%)

14 (93)

ANA Patterna, n (%)

 speckled

10 (67)

 homogeneous

4 (27)

 nucleolar

1 (7)

Scl-70

4 (27)

RNP

0

SSA/SSB

0

dsDNA

0

WBC (per μL), mean (SD)

7907 (2077)

ESRb, mean (SD), mm/h

15.4 (8.8)

Medication use, n (%)

 Antifibrotic agentc

  mycophenolate mofetil

12 (80)

  mycophenolic acid

2 (13)

  methotrexate

1 (7)

  cyclophosphamide

1 (7)

 prednisone

  (none)

9 (60)

  5 mg/d

3 (33)

  10 mg/d

1 (7)

 PPI

8 (53)

 CCB

4 (27)

 ACEi

5 (33)

 ARB

0

 statin

2 (13)

 ASA

5 (34)

 NSAID

1 (7)

Clinical symptoms, n (%)

 ILD

4 (27)

 SIBO

1 (7)

 GERD

9 (60)

 Hypothyroidism

2 (13)

 Raynaud’s

15 (100)

 DU

4 (27)

 GAVE

1 (7)

 Esophageal dysmotility

5 (33)

 Pericardial effusion

1 (7)

 SRC

1 (7)

 HTN

1 (7)

 Arthritis

2 (13)

 Sicca

1 (7)

 Calcinosis

1 (7)

 Intestinal Pseudobstruction

1 (7)

Disease duration months(SD)

 Raynaud’s

37 ± 40

 Skin sclerosis

19 ± 15

  1. ANA anti-nuclear antibody, ESR erythrocyte sedimentation rate, PPI proton pump inhibitor, CCB calcium channel blocker, ACEi angiotensin-converting enzyme inhibitor, ARB angiotensin receptor blocker, NSAID non-steroidal anti-inflammatory drug, ILD interstitial lung disease, SIBO small intestinal bacterial overgrowth, GERD gastroesophageal reflux disease, DU digital ulcer, GAVE gastric antral vascular ectasia, SRC scleroderma renal crisis, HTN hypertension
  2. a1 patient had both a speckled and nucleolar pattern; 1 patient did not have the presence of an ANA detected; 1 patient’s ANA status was unknown
  3. b2 patients had unknown levels of ESR at study entry
  4. c1 patient received both cyclophosphamide and mycophenolate mofetil