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Journal of Peking University (Health Sciences) ›› 2021, Vol. 53 ›› Issue (6): 1088-1093. doi: 10.19723/j.issn.1671-167X.2021.06.014

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Clinical and immunological characteristics of 88 cases of overlap myositis

XIAO Yun-shu1,ZHU Feng-yun-zhi2,LUO Lan2,XING Xiao-yan2,LI Yu-hui2,△(),ZHANG Xue-wu2,SHEN Dan-hua1   

  1. 1. Department of Pathology, Peking University People’s Hospital, Beijing 100044, China
    2. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
  • Received:2021-08-08 Online:2021-12-18 Published:2021-12-13
  • Contact: Yu-hui LI E-mail:liyuhui84@163.com
  • Supported by:
    National Natural Science Foundation of China(81801617);National Natural Science Foundation of China(81771678);National Natural Science Foundation of China(81971520);Peking University People’s Hospital Research and Development Funds(RDX2020-03)

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Abstract:

Objective: To investigate the clinical and immunological characteristics of overlap myositis (OM) patients. Methods: The data of 368 patients with idiopathic inflammatory myopathies (IIMs) admitted to Peking University People’s Hospital from January 2004 to August 2020 were analyzed retrospectively, including demographic characteristics, clinical characteristics (including fever, Gottron’s sign/papules, Heliotrope rash, V-sign, Shawl sign, Mechanic’s hands, skin ulceration, periungual erythema, subcutaneous calcinosis, dysphagia, myalgia, myasthenia, arthritis, Raynaud’s phenomenon, interstitial lung disease, pulmonary hypertension and myocardial involvement), laboratory characteristics, immunological characteristics [including antinuclear antibodies, rheumatoid factors, myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs)] and survival. The clinical and immunological characteristics and prognostic differences of OM and non-OM were compared. The Kaplan-Meier and Log Rank methods were used to analyze the survival. Results: A total of 368 patients were included. 23.9% (88/368) of IIMs patients were OM patients. Among the 88 OM patients, 85.2% (75/88) of them were female, and the median interval between disease onset and diagnosis was 13.5 months. The incidence of overlapped connective tissue diseases in the OM patients was dermatomyositis (DM) in 60.2%, polymyositis (PM) in 3.4%, immune-mediated necrotizing myopathy (IMNM) in 2.3% and anti-synthetase syndrome (ASS) in 34.1%. Compared with the non-OM patients, the proportion of the females in the OM patients was higher (85.2% vs. 72.1%, P=0.016), the OM patients had longer disease duration [13.5(4.5,48.0) months vs. 4.0(2.0,12.0) months, P<0.001]. As for clinical characteristics, compared with the non-OM patients, the incidence of V-sign (25.0% vs. 44.6%, P=0.001) and periungual erythema (8.0% vs. 19.6%, P=0.013) were lower; the incidence of Raynaud’s phenomenon (14.8% vs. 1.8%, P<0.001), interstitial pneumonia (88.6% vs. 72.1%, P=0.001), pulmonary hypertension (22.7% vs. 7.5%, P<0.001) and myocardial involvement (18.2% vs. 9.3%, P=0.033) were higher. As for immunological characteristics, compared with the non-OM patients, the incidence of elevated aspartate aminotransferase (AST) (31.8% vs. 45.0%, P=0.035) was lower and elevated C-reactive protein (CRP) (58.0% vs. 44.6%, P=0.037) was higher; the positive rates of antinuclear antibodies (ANA) (85.1% vs. 63.4%, P=0.001) and rheumatoid factors (RF) (40.2% vs. 17.8%, P<0.001) and anti-Ro-52 (71.6% vs. 56.1%, P=0.038) in serum were higher. There was no significant difference in the survival between the OM patients and non-OM patients. Conclusion: Pulmonary hypertension and myocardial involvement were frequently observed in OM.

Key words: Overlap myositis, Idiopathic inflammatory myopathy, Pulmonary hypertension, Autoantibodies

CLC Number: 

  • R593.26

Table 1

Clinical characteristics of patients with overlap myositis"

Variables OM (n=88) Non-OM (n=280) P (OM vs. non-OM)
Female,n (%) 75 (85.2) 202 (72.1) 0.016
Age on set/years,$\overline{x}$±s 50.3±14.4 49.2±14.0 0.525
Duration/months,M (P25,P75) 13.5 (4.5, 48.0) 4.0 (2.0, 12.0) <0.001
Fever,n (%) 40 (45.5) 107 (38.2) 0.262
Gottron’s sign /papules,n (%) 62 (70.5) 186 (66.4) 0.517
Heliotrope rash,n (%) 29 (33.0) 124 (44.3) 0.064
V-sign,n (%) 22 (25.0) 125 (44.6) 0.001
Shawl sign,n (%) 14 (15.9) 72 (25.7) 0.062
Mechanic’s hands,n (%) 35 (39.8) 88 (31.4) 0.156
Skin ulceration,n (%) 6 (6.8) 16 (5.7) 0.796
Periungual erythema,n (%) 7 (8.0) 55 (19.6) 0.013
Subcutaneous calcinosis,n (%) 1 (1.1) 7 (2.5) 0.686
Dysphagia,n (%) 4 (4.5) 5 (1.8) 0.226
Myalgia,n (%) 18 (20.5) 35 (12.5) 0.081
Myasthenia,n (%) 45 (51.1) 156 (55.7) 0.464
Arthritis,n (%) 48 (54.6) 125 (44.6) 0.113
Raynaud’s phenomenon,n (%) 13 (14.8) 5 (1.8) <0.001
Interstitial lung disease,n (%) 78 (88.6) 202 (72.1) 0.001
Pulmonary hypertension,n (%) 20 (22.7) 21 (7.5) <0.001
Myocardial involvement,n (%) 16 (18.2) 26 (9.3) 0.033

Table 2

Immunological characteristics of patients with overlap myositis"

Variables OM (n=88) Non-OM (n=280) P (OM vs. non-OM)
Elevated ALT,n (%) 32 (36.4) 131 (46.8) 0.109
Elevated AST,n (%) 28 (31.8) 126 (45.0) 0.035
Elevated LDH,n (%) 68 (77.3) 240 (85.7) 0.070
Elevated CK,n (%) 27 (30.7) 112 (40.0) 0.131
Elevated ESR,n (%) 54 (61.4) 145 (51.8) 0.141
Elevated CRP,n (%) 51 (58.0) 125 (44.6) 0.037
ANA positivity,n (%) 57/67 (85.1) 137/216 (63.4) 0.001
RF positivity,n (%) 33/82 (40.2) 43/241 (17.8) <0.001
Anti-Mi-2 positivity,n (%) 2/52 (3.9) 6/100 (6.0) 0.716
Anti-TIF-1γ positivity,n (%) 3/37 (8.1) 6/79 (7.6) 1.000
Anti-MDA5 positivity,n (%) 14/44 (31.8) 33/91 (36.3) 0.701
Anti-NXP2 positivity,n (%) 3/36 (8.3) 9/80 (11.3) 0.752
Anti-SAE1 positivity,n (%) 3/38 (7.9) 6/78 (7.7) 1.000
Anti-synthetase positivity,n (%) 34/60 (56.7) 66/118 (55.9) 1.000
Anti-SRP positivity,n (%) 8/54 (14.8) 7/99 (7.1) 0.156
Anti-Ku positivity,n (%) 6/55 (10.9) 4/100 (4.0) 0.168
Anti-PM-Scl100 positivity,n (%) 6/56 (10.7) 8/102 (7.8) 0.567
Anti-PM-Scl75 positivity,n (%) 4/50 (8.0) 9/96 (9.4) 1.000
Anti-Ro-52 positivity,n (%) 53/74 (71.6) 78/139 (56.1) 0.038

Figure 1

Kaplan-Meier survival analysis of OM and non-OM patients OM, overlap myositis."

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