Table 2 Clinical parameters of patients with stable MCTD phenotype and diagnostic converters at time point 1
Demographics | Stable MCTD phenotype N = 104 | Diagnostic converters N = 14 |
|---|---|---|
Age, years, mean (SD) | 43 (14) | 45 (13) |
Disease duration years, mean (SD) | 10 (9) | 9 (7) |
Male gender, N (%) | 27 (26) | 1 (7) |
Alarcon-Segovia’s criteria | 95 (91) | 13 (93) |
Kasukawa’s criteria | 89 (86) | 12 (86) |
Sharp’s criteria | 101 (97) | 13 (93) |
Interstitial lung disease, N (%) | 36 (36) | 3 (21) |
Cumulative frequency of clinical features | ||
Puffy hands, N (%) | 98 (94) | 10 (71)a |
Arthritis, N (%) | 79 (76) | 13 (93) |
Myositis, N (%) | 34 (33) | 2 (14) |
Raynaud’s phenomenon, N (%) | 103 (99) | 14 (100) |
Sclerodactily, N (%) | 30 (29) | 3 (21) |
Leukocytopenia, N (%) | 30 (29) | 7 (50) |
Thrombocytopenia, N (%) | 17 (16) | 0 |
Facial erythema, N (%) | 44 (42) | 6 (43) |
Pericarditis, N (%) | 10 (10) | 1 (7) |
Pleuritis, N (%) | 13 (13) | 1 (7) |
Pulmonary function tests | ||
Percentage DLCO pred, mean (SD) | 74 (17) | 80 (14) |
Percentage FVC pred, mean (SD) | 92 (18) | 99 (14) |
Genetics and anti-RNP antibodies at T1 | ||
Anti-RNP, U/mL, median (IQR) | 27 (5–66) | 104 (25–240) |
HLA DRB1*04:01, N (%) | 49 (50) | 4 (31) |