抗PM/Scl抗体相关风湿性疾病谱及其在特发性炎性肌病中的免疫学特征

doi:10.19723/j.issn.1671-167X.2025.06.002

Abstract

Abstract:

Objective: To systematically investigate the rheumatic disease spectrum associated with anti-PM/Scl antibodies and to clarify their clinical significance in idiopathic inflammatory myopathies (IIM). Methods: Patients who were tested positive for anti-PM/Scl antibodies by immunoblotting at Peking University People' s Hospital from January 2016 to December 2024 were enrolled. Clinical and immunolo gical data were systematically collected and compared across the subgroups defined by anti-PM/Scl75, anti-PM/Scl100, or dual antibody positivity. Results: A total of 422 anti-PM/Scl-positive patients were enrolled. Among them, 83.2% (351/422) were diagnosed with connective tissue disease (CTD), 7.8% (33/422) were not diagnosed with CTD, and 9.0% (38/422) had an undetermined clinical diagnosis. Among 422 patients, most commonly represented by IIM (19.7%), systemic sclerosis (SSc, 14.2%), overlap syndrome (11.8%), undifferentiated CTD (UCTD, 10.4%), rheumatoid arthritis (6.9%), Sjögren syndrome (6.4%), and systemic lupus erythematosus (6.2%), the remaining diseases accounting for 24.4%. Within the IIM subgroup, dermatomyositis predominated (74.7%), followed next by anti-synthetase syndrome (21.7%) and immune-mediated necrotizing myopathy (3.6%). Anti-PM/Scl75 antibodies were detected in 52.1% (220/422) of the total patients, anti-PM/Scl100 in 43.6% (184/422), and both in 4.3% (18/422). In the subsequent detailed analysis of the anti-PM/ Scl-positive subgroup, double-positive patients showed a significantly higher prevalence of SSc (38.9% vs. 14.1% vs. 12.0%, P=0.015) and interstitial lung disease (ILD, 70.6% vs. 28.8% vs. 35.4%, P=0.002) than those individuals with single antibody positivity alone. Raynaud phenomenon was observed more frequently in both the double-positive and anti-PM/Scl75-positive groups than in the anti-PM/Scl100-positive group (29.4% vs. 21.3% vs. 10.9%, P=0.007). The measured proportion of peri-pheral CD8⁺ T cells was also higher in double-positive patients (35.9%±14.1% vs. 30.4%±11.2% vs. 26.5%±9.7%, P= 0.008), whereas absolute regulatory T-cell levels were lower in the anti-PM/Scl75-positive group compared directly with the anti-PM/Scl100-positive group [7.6% (5.4%, 10.9%) vs. 9.0% (7.9%, 12.0%) vs. 8.8% (5.2%, 9.7%), P=0.017]. Additionally, co-positivity for anti-PM/Scl and other myositis- specific or myositis-associated antibodies was strongly associated with an increased frequency of ILD (P < 0.05). Conclusion: Anti-PM/Scl antibodies define a broad disease spectrum encompassing IIM, SSc, overlap syndromes, and UCTD. Dual positivity for anti-PM/Scl75 and anti-PM/Scl100 identifies patients prone to systemic sclerosis and pulmonary involvement, suggesting additive pathogenic effects of the two antibody specificities.

Key words: Anti-PM/Scl antibody, Idiopathic inflammatory myopathy, Connective tissue disease, CD8⁺ T cells

CLC Number:

R593.2

Yirui LIAN, Jingxuan LIU, Liang ZHAO, Jing ZHAO, Sitian ZANG, Yuhui LI. Rheumatic disease spectrum and immunological profile of anti-PM/Scl antibodies in idiopathic inflammatory myopathies[J].Journal of Peking University (Health Sciences), 2025, 57(6): 1018-1023.

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Table 3

References 20

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Variables	Anti-PM/Scl75 (+), n(%)	Anti-PM/Scl100 (+), n(%)	Anti-PM/Scl75/100 (+), n(%)	P value
Female	181 (82.3)	138 (75.0)	16 (88.9)	0.134^c
DM	29 (13.2)	27 (14.7)	6 (33.3)	0.077^c
IMNM	3 (1.4)	0 (0)	0 (0)	0.346^c
ASyS	8 (3.6)	10 (5.4)	0 (0)	0.560^c
SSc	31 (14.1)^a	22 (12.0)^a	7 (38.9)^b	0.015^c
Gottron sign	27/212 (12.7)	25/174 (14.4)	5/17 (29.4)	0.159^c
Heliotrope rash	17/211 (8.1)	19/174 (10.9)	0 (0)	0.377^c
Mechanic’s hands	25/211 (11.8)^a	17/174 (9.8)^a	6/17 (35.3)^b	0.018^c
Muscle weakness	38/211 (18.0)	30/174 (17.2)	5/17 (29.4)	0.425^c
Myalgia	49/209 (23.4)	42/171 (24.6)	5/17 (29.4)	0.787^c
Raynaud phenomenon	45/211 (21.3)^b	19/174 (10.9)^a	5/17 (29.4)^b	0.007^c
ILD	61/212 (28.8)^a	62/175 (35.4)^a	12/17 (70.6)^b	0.002^c
Arthritis	98/211 (46.4)	88/173 (50.9)	10/17 (58.8)	0.487
Elevated CK	41/200 (20.5)	29/154 (18.8)	1/16 (6.3)	0.461^c
ANA(+)	135/202 (66.8)	107/174 (61.5)	11/16 (68.8)	0.539
Anti-Jo-1(+)	10 (4.5)	6 (3.3)	0 (0)	0.810^c
Anti-PL7(+)	7 (3.2)	2 (1.1)	0 (0)	0.382^c
Anti-PL12(+)	6 (2.7)	6 (3.3)	0 (0)	0.869^c
Anti-Mi-2(+)	8 (3.6)	9 (4.9)	1 (5.6)	0.620^c
Anti-MDA5(+)	5 (2.3)	1 (0.5)	0 (0)	0.405^c
Anti-TIF1γ(+)	1 (0.5)	2 (1.1)	0 (0)	0.644^c
Anti-NXP2(+)	2 (0.9)	1 (0.5)	0 (0)	>0.999^c
Anti-SAE(+)	1 (0.5)	0 (0)	0 (0)	>0.999^c
Anti-SRP(+)	6 (2.7)	3 (1.6)	0 (0)	0.676^c
Anti-HMGCR(+)	1 (0.5)	0 (0)	0 (0)	>0.999^c
Anti-Scl70(+)	9 (4.1)	3 (1.6)	0 (0)	0.344^c
Anti-U1RNP(+)	12 (5.5)	5 (2.7)	0 (0)	0.337^c
Anti-Ro52(+)	39/194 (20.1)	20/161 (12.4)	2/14 (14.3)	0.147^c

Variables	Anti-PM/Scl 75(+) (n=220)	Anti-PM/Scl 100(+) (n=184)	Anti-PM/Scl 75/100(+) (n=18)	P value
Lymphocyte T/μL	1 064.0 (743.0, 1 389.0)	1 070.0 (694.0, 1 350.5)	808.0 (730.5, 1 762.3)	0.885
CD3⁺total T/%	73.3 (66.2, 82.0)^b	68.9 (60.1, 74.5)^a	78.9 (68.4, 84.2)^{a, b}	0.001
CD4⁺ helper T/μL	556.0 (357.0, 759.0)	528.0 (374.3, 824.5)	445.0 (295.0, 946.7)	0.899
CD4⁺helper T/%	40.0±11.6	37.6±10.0	39.2±11.9	0.346
CD8⁺cytotoxic T /μL	404.8 (235.5, 571.3)	360.7 (195.0, 525.4)	490.0 (289.0, 743.6)	0.396
CD8⁺cytotoxic T/%	30.4±11.2^b	26.5±9.7^a	35.9±14.1^b	0.008
CD19⁺B /μL	162.0 (74.0, 300.0)	196.4 (94.2，363.0)	149.0 (93.4，230.5)	0.378
CD19⁺B /%	11.5 (6.3, 15.6)	13.6 (8.1, 22.6)	11.0 (5.4, 15.8)	0.123
CD56⁺CD16⁺NK /μL	117.0 (65.0, 266.0)	197.0 (87.5, 297.0)	138.0 (84.9, 224.5)	0.099
CD56⁺CD16⁺NK/%	10.2 (4.6, 15.3)	13.0 (7.9, 19.1)	11.4 (4.8, 16.1)	0.062
pTfh/%	2.1 (1.4，3.1)	3.1 (1.7，4.0)	2.0 (1.0，3.1)	0.078
Treg/%	7.6 (5.4, 10.9)^a	9.0 (7.9, 12.0)^b	8.8 (5.2, 9.7)^{a, b}	0.017

Variables	Anti-PM/Scl(+) (n=348)	Anti-PM/Scl with MAAs/MSAs(+) (n=74)	P value
Female	276 (79.3%)	59 (79.7%)	0.539
Age of onset/years	51.1±15.9	48.9±17.5	0.296
Mechanic’s hands	33/332 (9.9%)	15/70 (21.4%)	0.009
Gottron sign	36/332 (10.8%)	21/71 (29.6%)	＜0.001
Shawl sign	13/329 (4.0%)	14/71 (19.7%)	＜0.001^a
V-sign	18/329 (5.5%)	15/71 (21.1%)	＜0.001
Skin ulceration	19/327 (5.8%)	8/71 (11.3%)	0.086^a
Myalgia	73/326 (22.4%)	23/71 (32.4%)	0.054
Muscle weakness	52/331 (15.7%)	21/71 (29.6%)	0.007
Pruritus	14/329 (4.3%)	11/71 (15.5%)	0.001^a
Skin hypercalcemia	4/329 (1.2%)	1/71 (1.4%)	0.626^a
Arthritis	161/331 (48.6%)	35/70 (50.0%)	0.470
Raynaud phenomenon	60/332 (18.1%)	9/70 (12.9%)	0.192
ILD	104/333 (31.2%)	31/71 (43.7%)	0.032
Elevated CK	54/304 (17.8%)	17/66 (25.8%)	0.095
CD3⁺total T/%	70.8±12.2	69.7±14.7	0.647
CD4⁺helper T/%	38.9±10.9	39.4±11.8	0.798
CD8⁺cytotoxic T/%	27.4 (21.7，36.4)	27.4 (18.2，37.4)	0.652
CD19⁺B /%	11.9 (6.8，17.4)	12.1 (9.0，20.5)	0.552
CD56⁺CD16⁺NK/%	11.5 (6.2，16.7)	9.1 (2.9，18.5)	0.181
Treg/%	8.5 (6.8，10.9)	7.2 (5.1，9.4)	0.114
pTfh/%	2.3 (1.5，3.5)	2.0 (1.3, 4.9)	0.663

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Rheumatic disease spectrum and immunological profile of anti-PM/Scl antibodies in idiopathic inflammatory myopathies

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