| All | PM | DM | ADM | OM | JM | P value |
---|---|---|---|---|---|---|---|
No. of patients no(%) | 188 | 41(21.8) | 100(53.2) | 13(6.9) | 27(14.4) | 7(3.7) | Â |
Died | 32 | 5 | 20 | 4 | 2 | 1 | NA |
Mortality (%) | 17.0 | 11.9 | 20 | 30.8 | 7.69 | 14.3 | NA |
Male: female ratio | 1:2.1 | 1:2.4 | 1:1.8 | 1:1.6 | 1:8 | 1:0.4 | NA |
Mean age ± S.D at onset (y) | 43.8(15.8) | 44.9(15.0) | 44.9(14.9) | 50 (13.8) | 42(16.8) | 15.7(2) | < 0.001†|
Complicated with ILD, n, % | 92(48.9) | 17(40.5) | 49(49.0) | 13(100.0) | 11(42.3) | 2(28.6) | 0.003 |
Complicated with PAH, n, % | 20(10.6) | 7(16.7) | 7(7.0) | 0(0.0) | 6(23.1) | 0(0.0) | > 0.05 |
Complicated with malignancy, n, % | 11(5.9) | 1(2.4) | 10(10.0) | 0(0.0) | 0(0.0) | 0(0.0) | > 0.05 |
Complicated with oropharyngeal dysphagia, n, % | 75(39.9) | 14(33.3) | 36(36.0) | 5(38.5) | 15(57.7) | 5(71.4) | > 0.05 |
Cardiac involvement, n, % | 64(34.0) | 14(33.3) | 37(37.0) | 4(30.8) | 4(15.4) | 5(71.4) | > 0.05 |
Mechanic hand at anytime, n, % | 28(14.9) | 1(2.4) | 21(21.0) | 3(23.1) | 3(11.5) | 0(0.0) | 0.038 |
Raynaud phenomenon at anytime, n, % | 29(15.4) | 5(11.9) | 10(10.0) | 3(23.1) | 11(42.3) | 0(0.0) | 0.001 |
Creatine kinase at initial visit in our center, mean (IQR) | 1440.9 (83, 1561.8) | 2106.8 (594.5, 2577.5) | 1117.4 (68.3, 971.3) | 67.9 (35, 93.5) | 1867.9 (236, 2452) | 3065.4 (114, 7908) | 0.013††|