Table 1.
Clinical variables
| Mean (SD) or as indicated | Inflammatory group N = 6 | Proliferative/noninflammatory group N = 9 | p value* |
|---|---|---|---|
| Clinical variables | |||
| Age | 58.7 (8.3) | 48.3 (7.7) | 0.03 |
| Sex, n (% women) | 5 (83 %) | 9 (100 %) | 0.40 |
| Ethnicity, n (% Caucasian) | 5 (83 %) | 5 (56 %) | 0.58 |
| BMI | 22.4 (2.6) | 24.5 (5.00) | 0.30 |
| Smoking, n (% past) | 4 (67 %) | 2 (22 %) | 0.14 |
| SSc subtype, n (% diffuse) | 3 (50 %) | 6 (67 %) | 0.62 |
| mRSS | 12.7 (12.6) | 16.6 (14.2) | 0.59 |
| GER symptoms, n (% present) | 5 (83 %) | 5 (56 %) | 0.58 |
| Dysphagia, n (% present) | 3 (50 %) | 3 (33 %) | 0.62 |
| Patulous esophagus HRCT, n (% present) | 5 (83 %) | 7 (78 %) | 1.00 |
| SSc disease duration (mo.) | 153.7 (132.5) | 83.7 (89.5) | 0.29 |
| GI symptom duration (mo.) | 61.8 (62.2) | 84.4 (87.0) | 0.57 |
| SSc autoantibodies, n (% positive) | N = 5 | ||
| • Scl-70 | 3 (50 %) | 3 (33 %) | 0.26 |
| • ACA | 1 (17 %) | 0 | |
| • RNA pol III | 1 (17 %) | 4 (44 %) | |
| • Negative | 0 | 2 (22 %) | |
| • Missing | 1 (17 %) | 0 | |
| Primary ANA pattern, n (% present) | |||
| • Centromere | 1 (17 %) | 0 | 0.08 |
| • Nucleolar | 1 (17 %) | 0 | |
| • Speckled | 1 (17 %) | 7 (78 %) | |
| • Homogenous | 3 (50 %) | 2 (22 %) | |
| + Mycophenolate, n (% current) | 1 (17 %) | 5 (56 %) | 0.29 |
| +Proton pump inhibition, N (% current) | 6 (100 %) | 9 (100 %) | N/A |
| FVC % predicted | 70.2 (23.9) | 88.9 (14.8) | 0.13 |
| TLC % predicted | 81.8 (20.2) | 100.6 (18.0) | 0.13 |
| DLCO % predicted | 46.5 (17.5) | 67.1 (20.3) | 0.06 |
| ILD present on HRCT, n (%) | 5 (83 %) | 7 (78 %) | 1.00 |
| Endoscopy, n (% present) | |||
| Esophagitis | 4 (67 %) | 5 (56 %) | 1.00 |
| Hiatal hernia | 5 (83 %) | 7 (78 %) | 1.00 |
| Pathology | |||
| Squamous epithelial lymphocytes | 10.0 (8.7) | 5.6 (5.9) | 0.36 |
| Basal cell hyperplasia, n (%) | N = 5 | N = 7 | |
| • 0 | 1 (17 %) | 3 (33 %) | 0.75 |
| • 1 | 3 (50 %) | 5 (56 %) | |
| • 2 | 1 (17 %) | 0 | |
| Degree of collagen deposition in lower esophageal biopsies, n (%) | N = 4 | N = 4 | |
| • 0 | 2 (33 %) | 4 (44 %) | 0.38 |
| • 1 | 1 (17 %) | 0 | |
| • 2 | 1 (17 %) | 0 | |
| Candida esophagitis, n (%) | 2 (33 %) | 0 | 0.14 |
Only patients with SSc were included in these analyses. Subject SSc12 was excluded because the upper esophageal biopsy was influenced by technical artifact that precluded intrinsic subset assignment
BMI body mass index, SSc systemic sclerosis, mRSS modified Rodnan skin score, GER gastroesophageal reflux, HRCT high-resolution computed tomography, GI gastrointestinal, Scl-70 anti-topoisomerase I, ACA anticentromere, RNA pol III anti-RNA polymerase III antibodies, ANA antinuclear antibodies, N/A not applicable, FVC forced vital capacity, TLC total lung capacity, DLCO diffusion capacity for carbon monoxide percent predicted, ILD interstitial lung disease
* t test for continuous variables and Fisher’s exact test for categorical variables comparing inflammatory to proliferative/noninflammatory groups