Characteristics | Fibrotic (n = 10) | Atrophic (n = 6) |
---|---|---|
Mean age (range) | 54 (39–72) | 58 (37–69) |
Mean disease duration, months (range) | 11 (4–18) | 96 (36–168) |
Male/female | 2/8 | 0/6 |
Organ involvement | Â | Â |
Mean skin score (range) | 33 (19–41) | 17 (11–24) |
Oesophageal | 7/10 | 3/6 |
Other gastrointestinal | 4/10 | 1/6 |
Lung | 4/10 | 2/6 |
Muscle | 3/10 | 0/6 |
Renal | 2/10 | 1/6 |
Cardiac | 0/10 | 1/6 |
Pulmonary hypertension | 2/10 | 0/6 |
Serology | Â | Â |
Antinuclear | 10/10 | 6/6 |
Anti-topoisomerase 1 | 4/10 | 3/6 |
Anti-RNA polymerase I/III | 2/10 | 1/6 |
Anti-nuclear RNP | 1/10 | 1/6 |
Microvascular damage | Â | Â |
Structural capillary damage | 10/10 | 6/6 |