临床无肌病性皮肌炎与皮肌炎临床及免疫学特征比较

doi:10.19723/j.issn.1671-167X.2020.06.003

Abstract

Abstract:

Objective: To study the differences between clinically amyopathic dermatomyositis (CADM) and typical dermatomyositis (DM) on clinical and immunological features. Methods: By collecting clinical data of 106 CADM patients and 158 DM patients from January 2010 to June 2019 in the department of Rheumatology and Immunology, Peking University People’s Hospital, the clinical characteristics and immunological features in the two groups were compared, and the distribution characters and the clinical meanings of myositis autoantibodies were discussed in the two groups respectively. Myositis autoantibodies were measured by immunoblotting according to the manufacturers’ instructions. Results: In the aspects of clinical manifestations, CADM presented more with onset of interstial lung diseases (ILD) compared with DM (20.7% vs. 7.6%, P=0.002), and CADM-ILD was more likely to be acute ILD (58.3% vs. 26%, P<0.001), and there were no differences between CADM and DM in cutaneous manifestations, accompanied with connective tissue disease (CTD) and malignancy. In CADM, the positive rate of rheumatoid factors and antinuclear antibodies was lower in DM. The most common myositis specific autoantibodies (MSAs) in CADM were anti-MDA5 (36%), anti-PL-7 (11.2%) and anti-TIF-1γ (10.1%). The most common MSAs in DM were anti-Jo-1 (19.2%), anti-TIF-1γ (11.5%) and anti-MDA5 (11.5%). Anti-MDA5 was correlated with acute ILD and skin ulceration both in CADM and DM; in CADM, skin ulceration was not associated with the titer of anti-MDA5; while in DM, skin ulceration was associated with high titer of anti-MDA5. In DM, anti-TIF-1γ was correlated with heliotrope eruption, V/shawl neck sign, perionychia erythma and malignancy, and higher rate of malignancy was seen in all titers of the anti-TIF-1γ positive patients. In CADM, anti-TIF1-γ showed no correlation with clinical manifestations. The most common myositis associated autoantibody was anti-Ro-52 both in CADM and DM. In CADM, anti-Ro-52 was associated with Raynaud’s phenomenon and chronic ILD, while in DM, anti-Ro-52 was associated with mechanic’s hands, noninfectious fever and accompanied CTD. Conclusion: Compared with DM, ILD is more likely to be acute in CADM. It is different between CADM and DM about the distribution of myositis autoantibodies and the clinical significance of the same myositis antibody, and the clinical significance of some myositis antibodies is related to titers.

Key words: Clinically amyopathic dermatomyositis, Dermatomyositis, Myositis autoantibody

CLC Number:

R593.26

Yu-zhou GAN,Yu-hui LI,Li-hua ZHANG,Lin MA,Wen-wen HE,Yue-bo JIN,Yuan AN,Zhan-guo LI,Hua YE. Comparison of clinical and immunological features between clinically amyopathic dermatomyositis and typical dermatomyositis[J].Journal of Peking University (Health Sciences), 2020, 52(6): 1001-1008.

Figures/Tables 6

Table 1

Comparison of clinical manifestations and laboratory parameters between CADM and DM"

Items	CADM (n=106)	DM (n=158)	F/χ²	P
Age/years, $x - ± s$	50.47±12.44	51.63±13.85	0.484	0.487
Gender, female:male (%female)	82:24 (77.4)	109:49 (69.0)	2.222	0.136
Age of onset/years, $x - ± s$	48.84±13.07	48.92±14.79	0.001	0.969
Cutaneous manifestation
Gottron’s sign/papule, n(%)	85 (80.2)	115 (72.8)	1.894	0.169
Mechanic’s hands, n(%)	41 (38.7)	56 (35.4)	0.286	0.593
Heliotrope eruption, n(%)	59 (55.7)	82 (51.9)	0.361	0.548
V shawl neck sign, n(%)	59 (55.7)	92 (58.2)	0.171	0.679
Skin ulceration, n(%)	13 (12.3)	17 (10.8)	0.143	0.706
Perionychia erythma, n(%)	21 (19.8)	35 (22.2)	0.208	0.648
Skin calcinosis, n(%)	7 (6.6)	8 (5.1)	0.281	0.596
Pulmonary involvement
ILD, n(%)	84 (79.2)	131 (82.9)	0.564	0.453
Acute, n(%)	49 (58.3)	34 (26.0)	22.640	<0.001
Asymptomatic, n(%)	17 (20.2)	43 (32.8)	4.030	0.045
ILD onset before CADM diagnosed, n(%)	22 (20.7)	12 (7.6)	9.792	0.002
Systemic symptoms
Noninfectious fever, n(%)	41 (38.7)	56 (35.4)	0.286	0.593
Arthralgia, n(%)	56 (52.8)	65 (41.1)	3.493	0.062
Raynaud phenomenon, n(%)	12 (11.3)	22 (13.9)	0.383	0.536
Splenomegaly, n(%)	11 (10.4)	10 (6.3)	1.383	0.240
Weight loss, n(%)	38 (35.8)	44 (27.8)	1.897	0.168
Accompanied CTD, n(%)	20 (18.9)	31 (19.6)	0.023	0.879
Accompanied malignancy, n(%)	5 (4.7)	14 (8.9)	1.631	0.202
Serum TAAs
CEA/(μg/L), M(P₂₅, P₇₅)	2.89 (1.76, 5.27)	2.00 (1.10, 4.03)	2.750	0.006
AFP/(μg/L), M(P₂₅, P₇₅)	2.45 (1.89, 3.17)	2.45 (1.78, 3.46)	0.297	0.767
CA19-9/(U/mL), M(P₂₅, P₇₅)	11.81 (4.97, 16.53)	9.92 (5.78, 18.87)	0.471	0.638
CYFRA21-1/(μg/L), M(P₂₅, P₇₅)	3.38 (2.05, 5.49)	3.06 (2.36, 4.81)	0.260	0.795
NSE/(μg/L), M(P₂₅, P₇₅)	14.08 (11.15, 17.20)	16.20 (12.39, 23.47)	2.848	0.004
Immunological parameters
RF positive, n(%)^*	11 (10.8)	32 (20.5)	4.203	0.040
ANA (≥1:80), n(%)^*	36 (35.3)	78 (50.0)	6.538	0.011

Table 1

Table 2

Comparison of distribution of myositis autoantibodies between CADM and DM"

Items	CADM (n=89)				DM (n=130)				Comparison by overall positive rate		Comparison by titers
Items	Overall	+	++	+++	Overall	+	++	+++	χ²	P	χ²	P
Myositis specific autoantibodies (MSAs)
Mi-2α	1 (1.1)	1 (1.1)	0	0	7 (5.4)	3 (2.3)	3 (2.3)	1 (0.8)	-	0.146	3.124	0.077
Mi-2β	4 (4.5)	3 (3.4)	0	1 (1.1)	6 (4.6)	3 (2.3)	3 (2.3)	0	-	1	0.001	0.970
TIF-1γ	9 (10.1)	8 (9.0)	1 (1.1)	0	15 (11.5)	3 (2.3)	5 (3.8)	9 (6.9)	0.444	0.505	4.202	0.040
MDA5	32 (36.0)	6 (6.7)	6 (6.7)	20 (22.5)	15 (11.5)	5 (3.8)	2 (1.5)	8 (6.2)	20.65	<0.001	18.697	<0.001
NXP2	5 (5.6)	2 (2.2)	1 (1.1)	2 (2.2)	7 (5.4)	1 (0.8)	3 (2.3)	3 (2.3)	0.006	0.941	0.022	0.883
SAE1	3 (3.4)	0	0	3 (3.4)	1 (0.8)	0	0	1 (0.8)	-	0.306	-	0.306
SRP	4 (4.5)	4 (4.5)	0	0	8 (6.2)	2 (1.5)	2 (1.5)	4 (3.1)	-	0.765	2.108	0.147
Jo-1	7 (7.9)	2 (2.2)	2 (2.2)	3 (3.4)	25 (19.2)	2 (1.5)	3 (2.3)	20 (15.4)	5.47	0.019	7.255	0.007
PL-7	10 (11.2)	5 (5.6)	3 (3.4)	2 (2.25)	10 (7.7)	2 (1.5)	4 (3.1)	4 (3.1)	0.799	0.371	0.067	0.796
PL-12	7 (7.9)	1 (1.1)	1 (1.1)	5 (5.6)	7 (5.4)	3 (2.3)	0	4 (3.1)	0.543	0.461	1.034	0.309
EJ	0 (0.0)	0	0	0	10 (7.7)	1 (0.8)	1 (0.8)	8 (6.2)	-	0.006	6.744	0.009
OJ	2 (2.2)	2 (2.25)	0	0	4 (3.1)	3 (2.3)	1 (0.8)	0	-	1	0.336	0.562
Myositis associated autoantibodies (MAAs)
PM-Scl100	6 (6.7)	5 (5.6)	1 (1.12)	0	5 (3.8)	1 (0.8)	1 (0.8)	3 (2.3)	0.928	0.335	0.055	0.815
PM-Scl75	6 (6.7)	3 (3.4)	1 (1.12)	2 (2.2)	9 (6.9)	2 (1.5)	4 (3.1)	3 (2.3)	0.003	0.958	0.089	0.766
Ku	9 (10.1)	4 (4.5)	2 (2.2)	3 (3.4)	4 (3.1)	2 (1.5)	1 (0.8)	1 (0.8)	-	0.041	4.218	0.040
Ro-52	44 (49.4)	8 (9.0)	5 (5.6)	31 (34.8)	70 (53.8)	4 (3.1)	4 (3.1)	62 (47.7)	0	1	1.952	0.162
Both MSAs and MAAs positive	79 (88.8)	-	-	-	112 (86.1)	-	-	-	1.143	0.285	-	-
Only MSAs positive	62 (69.7)	-	-	-	99 (76.1)	-	-	-	0	0.989	-	-
Only MAAs positive	54 (60.1)	-	-	-	79 (60.8)	-	-	-	0.323	0.570	-	-

Table 2

Table 3

Table 4

Comparison of the correlation of myositis autoantibodies and different types of ILD between CADM and DM"

Items	CADM				DM
Items	Non-ILD (n=16)	Acute ILD (n=44)	Chronic ILD (n=29)	P	Non-ILD (n=22)	Acute ILD (n=27)	Chronic ILD (n=81)	P
TIF-1γ	3 (18.7)	5 (11.4)	1 (3.4)	0.234	8 (36.4)	0^*	9 (11.1)^*	0
MDA5	3 (18.7)	22 (50.0)^*	7 (24.1)^§	0.027	0	8 (29.6)^*	7 (8.6)^§	0.002
Jo-1^※	-	-	-	-	1 (4.5)	7 (25.9)^*	17 (21.0)^*	0.039
Ro-52	4 (25.0)	21 (47.7)	19 (65.5 $) * §$	0.018	9 (40.9)	17 (63.0)	44 (54.3)	0.415

Table 4

Figure 1

Figure 2

References 33

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Items	CADM						DM
	TIF-1γ		MDA5		Ro-52		TIF-1γ		MDA5		Ro-52
	r	P	r	P	r	P	r	P	r	P	r	P
Mechanic’s hands	-0.058	0.524	0.117	0.275	0.101	0.344	0.004	0.961	0.058	0.509	0.196	0.026
Heliotrope eruption	0.025	0.818	0.165	0.123	-0.335	0.001	0.310	<0.001	0.146	0.098	-0.121	0.171
V/shawl neck sign	0.111	0.302	0.014	0.893	-0.157	0.141	0.280	0.001	0.127	0.151	-0.256	0.003
Skin ulceration	-0.013	0.907	0.289	0.006	-0.167	0.117	-0.022	0.805	0.310	<0.001	0.013	0.882
Perionychia erythma	0.017	0.875	0.115	0.281	-0.148	0.167	0.287	<0.001	0.300	0.001	0.024	0.783
Skin calcinosis	-0.078	0.465	-0.169	0.112	0.063	0.560	-0.087	0.323	-0.079	0.372	-0.179	0.042
Noninfectious fever	0.012	0.912	-0.015	0.892	0.094	0.381	-0.238	0.006	-0.082	0.354	0.257	0.003
Arthralgia	-0.156	0.144	0.107	0.317	0.102	0.343	-0.165	0.060	0.204	0.020	0.141	0.109
Raynaud’s phenomenon	-0.013	0.907	-0.106	0.322	0.246	0.020	-0.046	0.601	-0.025	0.775	0.052	0.556
Malignancy	-0.070	0.516	-0.022	0.840	-0.078	0.465	0.476	<0.001	-0.124	-0.160	-0.102	0.247
Accompanied CTD	-0.089	0.407	0.073	0.498	0.066	0.540	-0.135	0.124	-0.110	0.214	0.329	<0.001

Comparison of clinical and immunological features between clinically amyopathic dermatomyositis and typical dermatomyositis

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