Age at onset (years), mean (SD) | 38 (17) | 46 (15) |
Sex (male:female) | 4:21 | 5:37 |
Duration (years), mean (SD) | 5.1 (10.5) | 7.7 (7.1) |
| | |
Clinical features
| | |
Pitting scars or skin ulcers | 80* | 38 |
Short sublingual frenulum | 48 | 59 |
Contracture of phalanges | 57 | 41 |
Diffuse pigmentation | 52 | 50 |
| | |
Organ involvement
| | |
Lung | | |
Pulmonary fibrosis | 64* | 24 |
Pulmonary hypertension | 21 | 18 |
Decreased %VC | 64* | 24 |
Decreased %Tlco | 84* | 40 |
Oesophagus | 67 | 77 |
Heart | 19 | 11 |
Kidney | 0 | 2 |
Joint | 24 | 24 |
Muscle | 24 | 17 |
| | |
Laboratory findings
| | |
Antitopoisomerase I antibody | 48 | 29 |
Anticentromere antibody | 32 | 43 |
Anti-U1RNP antibody | 8 | 7 |
Rheumatoid factor | 29 | 13 |
Raised ESR | 62* | 26 |
Raised CRP | 33 | 20 |
Increased IgG | 68 | 45 |