Tables 1 to 4 summarized the characteristics and study results of all 136 patients. Among them, 99 (73%) patients were female and 37 (27%) were male. 78 (57%) patients were diagnosed between the ages of 40 and 60. 72 patients were recorded whether felt itch or not during the disease. Rash (n=55, 40%) was the most common first symptoms of DM, followed by respiratory symptom (n=30, 22%), arthritis (n=27, 20%), myasthenia (n=13, 10%), and atypical symptom (n =11, 8%). Atypical symptom included fever (n=7), diarrhea (n=2), dysphagia (n=1) and oral ulcer (n=1).
3.1 General features
The comparison of general features by clinical subgroups was shown in Table 1. The female to male ratio was about 2.7:1 in total patients. 43% (31/72) patients experienced itchy skin during the disease. There were no significant differences in gender, age, BMI, delay in diagnosis, smoking and itch (P > 0.05). The incidence of ILD in respiratory symptom groups was the higher than rash groups and atypical groups (100% vs 60%, 100% vs 54.4%, P < 0.05).
Table 1. General features in different subgroups of DM
Factors
|
Rash
(N=55)
|
Myasthenia
(N=13)
|
Arthritis
(N=27)
|
Respiratory
(N=30)
|
Atypical
(N=11)
|
Test statistic
|
P
|
Gender, female (%)
|
41 (74.5)
|
8 (61.5)
|
21 (77.8)
|
22 (73.3)
|
7 (63.6)
|
1.726
|
0.796
|
Age(year)
|
47
(39, 61)
|
48
(39, 56)
|
50
(39, 54)
|
53
(47, 57)
|
63
(46, 65)
|
5.679
|
0.224
|
Delay in diagnosis (months)
|
3.5
(2.0, 6.3)
|
5
(2.5, 9.5)
|
3
(2, 9)
|
3
(2, 5)
|
2
(1, 4)
|
4.158
|
0.385
|
BMI (kg/m2)
|
23.7
(21.8, 26.2)
|
23.0
(19.3, 25.9)
|
23.4
(20.8, 25.4)
|
25.5
(21.7, 26.6)
|
22.5
(21.7, 26.7)
|
2.975
|
0.562
|
Smoking (%)
|
5 (9.1)
|
1 (7.7)
|
1 (3.7)
|
2 (6.7)
|
2 (18.2)
|
2.522
|
0.637
|
ILD (%)
|
33 (60.0) a
|
10 (76.9) ab
|
21 (77.8) ab
|
30 (100.0) b
|
6 (54.4) a
|
18.337
|
0.001
|
Itch (%) #
|
17 (17/31, 54.8)
|
3 (3/5, 60.0)
|
5 (5/13, 38.5)
|
5 (5/18, 27.8)
|
1 (1/5, 20.0)
|
5.096
|
0.273
|
BMI = Body Mass Index, ILD = interstitial lung disease.
Note: # 72 patients were recorded whether felt itch or not during the disease. Qualitative variables were presented as counts (n) and percentages (%) by chi-square test or Fisher's Exact test. Values with letters a, b and significantly different across columns with Bonferroni's comparison tests (P < 0.05). Quantitative variables were presented as median (P25, P75) By Kruskal-Wallis test.
3.2 Laboratory indicators
serological indicators
We examined the serological indicators in these five groups (Table 2). There were differences in the distribution of TG in the DM subgroups by Kruskal-Wallis tests (P = 0.029), but no statistic difference was found in pairwise comparison with Bonferroni’s multiple tests. The differences in other serological indicators among different subgroups were no statistical significance (P > 0.05).
Table 2. Serological indicators in different subgroups of DM
Factors
|
Rash
(N=55)
|
Myasthenia
(N=13)
|
Arthritis
(N=27)
|
Respiratory
(N=30)
|
Atypical
(N=11)
|
Test statistic
|
P
|
CK (U/L)
|
63
(42, 230)
|
94
(48, 168)
|
31
(23, 87)
|
68
(39, 119)
|
86
(27, 185)
|
8.838
|
0.065
|
LDH (U/L)
|
344
(261, 476)
|
370
(320, 457)
|
297
(254, 350)
|
369
(269, 460)
|
321
(268, 418)
|
6.236
|
0.182
|
TC (mmol/L)
|
4.1
(3.6, 5.1)
|
5.1
(4.1, 5.9)
|
4.0
(3.5, 5.0)
|
4.3
(3.9, 5.3)
|
3.7
(3.3, 4.5)
|
8.934
|
0.063
|
TG (mmol/L)
|
1.6
(1.1, 2.1)
|
2.5
(1.5, 5.4)
|
2.2
(1.5, 3.2)
|
1.7
(1.3, 2.3)
|
1.2
(1.0, 1.7)
|
10.776
|
0.029
|
HDL (mmol/L)
|
1.1 ±0.4
|
1.0 ±0.3
|
1.0 ±0.3
|
1.0 ±0.3
|
0.9 ±0.1
|
1.805
|
0.132
|
LDL (mmol/L)
|
2.6 ±0.9
|
2.9 ±0.7
|
2.5 ±1.0
|
2.9 ±1.0
|
2.4 ±0.5
|
1.164
|
0.330
|
KL-6 (U/mL)
|
653
(349, 1223)
|
919
(416, 1400)
|
899
(353, 1434)
|
885
(708, 1203)
|
685
(249, 1046)
|
4.288
|
0.368
|
ferritin(ng/mL)
|
625.4
(192.3, 1073.0)
|
877.0
(93.1, 983.1)
|
503.0
(195.3, 680.0)
|
587.0
(253.2, 927.7)
|
789
(543, 789)
|
1.809
|
0.771
|
CD4/8
|
1.6
(1.2, 2,7)
|
1.2
(1.0, 3.2)
|
2.3
(1.7, 2.6)
|
1.4
(1.1, 1.9)
|
2.3
(1.3, 3.0)
|
6.334
|
0.176
|
CK = creatine kinase, LDH = lactate dehydrogenase, TC = total cholesterol, TG = triglyceride, HDL = high-density lipoprotein, LDL = low-density lipoprotein, KL-6 = krebs von den lungen-6.
Note: Qualitative variables were presented as counts (n) and percentages (%) by chi-square test or Fisher's Exact test. Quantitative variables were presented as median (P25, P75) by Kruskal-Wallis test or mean ± SD by one-way ANOVA.
PFTs
80 patients did the PFTs in our study (Table 3). FEV1/FVC in myasthenia group was significantly higher than that in respiratory symptom group (88.2 (81.8, 93.9) vs 81.4 (80.1, 83.7), P < 0.05). MMEF75/25 in respiratory symptom group was significantly lower than that in myasthenia group and arthritis group (45.3 (34.5, 54.0) vs 74.2 (57.2, 99.5), P < 0.05). FVC in respiratory symptom group was significantly lower than that in rash group, arthritis group and atypical group (67.2 ± 15.8 vs 82.9 ± 17.6, 67.2 ± 15.8 vs 78.4 ± 15.6, 67.2 ± 15.8 vs 86.8 ± 11.6, P < 0.05), and DLCO in respiratory symptom group was also significantly lower than that in rash group, arthritis group and atypical group (44.6 ± 12.3 vs 62.8 ± 16.8, 44.6 ± 12.3 vs 57.9 ± 13.0, 44.6 ± 12.3 vs 67.0 ± 15.5, P < 0.05).
Table 3. PFTs in different subgroups of DM
Factors
|
Rash
|
Myasthenia
|
Arthritis
|
Respiratory
|
Atypical
|
Test statistic
|
P
|
FEV1/FVC
|
81.0ab
(77.5, 86.3)
|
88.2a
(81.8, 93.9)
|
84.4ab
(79.1, 87.7)
|
81.4b
(80.1, 83.7)
|
82.6ab
(67.9, 85.4)
|
10.336
|
0.035
|
MMEF75/25
|
59.1ab
(46.5, 70.5)
|
74.2a
(57.2, 99.5)
|
76.0a
(48.5, 87.5)
|
45.3b
(34.5, 54.0)
|
65.6ab
(31.7, 113.6)
|
18.234
|
0.001
|
FVC
|
82.9 ±17.6a
|
78.4 ± 15.6ab
|
85.7 ± 18.4a
|
67.2 ± 15.8b
|
86.8 ± 11.6a
|
4.085
|
0.005
|
DLCO
|
62.8 ±16.8a
|
53.7 ±15.2ab
|
57.9 ± 13.0a
|
44.6 ± 12.3b
|
67.0 ± 15.5a
|
5.560
|
0.001
|
FEV1/FVC = forced expiratory volume in 1s/Forced vital capacity, MMEF75/25 = percentage of maximum mid-expiratory flow to estimated value, FVC =percentage of forced vital capacity to estimated value, DLCO = percentage of diffusion capacity for carbon to estimated value.
Note: Quantitative Variables were presented as median (P25, P75) By Kruskal-Wallis test or mean ± SD by one- way ANOVA. Values with letters a, b and significantly different across columns with Bonferroni's comparison tests (P < 0.05).
Myositis antibodies
The differences in myositis antibodies among subgroups included anti-TIF1γ and anti-RO52 antibodies (Table 4). The positive rate of anti-TIF1γ antibodies in rash group was significantly higher than that in arthritis group and respiratory symptom group (29.1% vs 0.0%, 29.1% vs 0.0%, P < 0.05). Atypical group had a higher positive rate of anti-TIF1γ antibodies than arthritis group and respiratory symptom group (27.3% vs 0.0%, 27.3% vs 0.0%, P < 0.05). The positive rate of anti-RO52 antibodies in respiratory symptom group was significantly higher than that in rash group and myasthenia group (83.3% vs 49.1%, 83.3% vs 30.8%, P < 0.05). Arthritis group had a higher positive rate anti-RO52 antibodies than that in rash group and myasthenia group (85.2% vs 49.1%, 85.2% vs 30.8%, P < 0.05). There were no significant differences in other myositis antibodies (P > 0.05).
Table 4. Myositis antibodies in different subgroups of DM
Antibodies
|
Rash
(N = 55)
(n/N, %)
|
Myasthenia
(N = 13)
(n/N, %)
|
Arthritis
(N = 27)
(n/N, %)
|
Respiratory
(N = 30)
(n/N, %)
|
Atypical
(N = 11)
(n/N, %)
|
P
|
MDA5
|
24 (43.6)
|
7 (53.8)
|
20 (74.1)
|
17 (56.7)
|
4 (36.4)
|
0.208
|
TIF1γ
|
16 (29.1) a
|
2 (15.4) ab
|
0 (0.0) b
|
0 (0.0) b
|
3 (27.3) a
|
0.000
|
Mi-2
|
4 (7.3)
|
1 (7.7)
|
0 (0.0)
|
1 (3.3)
|
1 (9.1)
|
0.646
|
RO52
|
27 (49.1) a
|
4 (30.8) a
|
23 (85.2) b
|
25 (83.3) b
|
6 (54.5) ab
|
0.000
|
MDA5 = anti-melanoma differentiation-associated protein 5, TIF1γ = anti-transcription intermediary factor 1-gamma, Mi-2 = anti-chromodomain-helicase-DNA-binding proteins.
Note: Qualitative variables were presented as counts (n) and percentages (%) by chi-square test or Fisher's Exact test. Values with letters a, b and significantly different across columns with Bonferroni's comparison tests (P < 0.05).
3.3 Survival analysis
All patients were followed up in our study, and six patients died. The longest follow-up time was 54 months, and the mean follow-up time was 20 months. Kaplan-Meier analysis was used to analyze survival curves among different subgroups (Figure 1). The 1-year and 3-year survival rate of rash groups and atypical symptom groups were 100%. The 1-year and 3-year survival rates of myasthenia groups were both 84.6%. The 1-year and 3-year survival rates of respiratory symptoms groups were both 96.7%. The 1-year and 3-year survival rates of arthritis groups were 96.3% and 88.9%. There were significant differences in cumulative survival rate among the five groups by Log-rank test (P = 0.048, P < 0.05). The survival rate of rash groups was higher than myasthenia groups (P = 0.003, P < 0.05) and arthritis groups (P = 0.018, P < 0.05). There were no significant differences in cumulative survival rate between other groups (P > 0.05).