Acta neurologica Taiwanica · 2025
The Characteristics of Imaging Patterns on Whole-body Magnetic Resonance Imaging of Anti-signal Recognition Particle Antibody-positive Immune-mediated Necrotizing Myopathy.
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Hou Ya-Yuan, Huang Wei-Chin, Huang Chi-Ren, Chen Shun-Sheng, Tsai Nai-Wen, Chen Shu-Fang
Аннотация
BACKGROUND: Immune-mediated necrotizing myopathy (IMNM) is a rapidly progressive subtype of inflammatory myositis that can be managed with early immunotherapy. Anti-signal recognition particle (anti-SRP)-positive IMNM is frequently associated with severe muscle weakness and respiratory complications, while whole-body muscle MRI involvement remains insufficiently characterized.
OBJECTIVES: The aim of this study was to identify the characteristics of muscle magnetic resonance imaging (MRI) of patients with immune-mediated necrotizing myopathy (IMNM) with anti-signal recognition particle (SRP) antibody and to further evaluate the correlation between these MRI features and clinical presentation.
MATERIALS AND METHODS: We identified 16 patients with anti-SRP myopathy, the diagnosis of IMNM was confirmed by muscle biopsy, and the whole-body MRI was performed in all 16 patients. Clinical information and laboratory data were collected. The severity of the short-T1 inversion recovery (STIR) sequences was classified into three grades (0, 0.5, and 1) according to the pattern and intensity of individual muscle involvement. Serial muscle group grading was performed using Friedman's test, and the correlation between MRI STIR pattern and clinical data was calculated using Spearman's rank correlation coefficient.
RESULTS: The 16 patients included 9 women and 7 men, with an average age of 43.4. The mean time-to-onset was 5.5 months. Whole-body muscle MRI STIR sequencing revealed muscle edema in all tested patients. Marked edema was noted in the pelvis, bilateral shoulders, posterior compartment of the arm, and concentric muscles of the shoulder and pelvis (all P < 0.001). Correlation analysis indicated that higher muscle STIR signaling was correlated with higher values of erythrocyte sedimentation rate, SRP antibody titer, and compound motor action potential (CMAP) reduction. In addition, we observed lower values of C-reactive protein, forced vital capacity (FVC), ejection fraction, clinical pretreatment with steroids (premedication steroids), and Medical Research Council scores in specific muscles. Reduced FVC was further found to correlate with edema in the muscles of the cervical spine, shoulder, anterior forearm, and pelvis, leading to reduced respiratory function.
CONCLUSIONS: Whole-body MRI in anti-SRP patients revealed a characteristic edema pattern with an affinity for specific muscles of the shoulders and pelvis with a radial distribution, with edema severity corresponding to clinical severity, particularly respiratory function. Pretreatment with steroids reduced edema, indicating the efficacy of this treatment.
OBJECTIVES: The aim of this study was to identify the characteristics of muscle magnetic resonance imaging (MRI) of patients with immune-mediated necrotizing myopathy (IMNM) with anti-signal recognition particle (SRP) antibody and to further evaluate the correlation between these MRI features and clinical presentation.
MATERIALS AND METHODS: We identified 16 patients with anti-SRP myopathy, the diagnosis of IMNM was confirmed by muscle biopsy, and the whole-body MRI was performed in all 16 patients. Clinical information and laboratory data were collected. The severity of the short-T1 inversion recovery (STIR) sequences was classified into three grades (0, 0.5, and 1) according to the pattern and intensity of individual muscle involvement. Serial muscle group grading was performed using Friedman's test, and the correlation between MRI STIR pattern and clinical data was calculated using Spearman's rank correlation coefficient.
RESULTS: The 16 patients included 9 women and 7 men, with an average age of 43.4. The mean time-to-onset was 5.5 months. Whole-body muscle MRI STIR sequencing revealed muscle edema in all tested patients. Marked edema was noted in the pelvis, bilateral shoulders, posterior compartment of the arm, and concentric muscles of the shoulder and pelvis (all P < 0.001). Correlation analysis indicated that higher muscle STIR signaling was correlated with higher values of erythrocyte sedimentation rate, SRP antibody titer, and compound motor action potential (CMAP) reduction. In addition, we observed lower values of C-reactive protein, forced vital capacity (FVC), ejection fraction, clinical pretreatment with steroids (premedication steroids), and Medical Research Council scores in specific muscles. Reduced FVC was further found to correlate with edema in the muscles of the cervical spine, shoulder, anterior forearm, and pelvis, leading to reduced respiratory function.
CONCLUSIONS: Whole-body MRI in anti-SRP patients revealed a characteristic edema pattern with an affinity for specific muscles of the shoulders and pelvis with a radial distribution, with edema severity corresponding to clinical severity, particularly respiratory function. Pretreatment with steroids reduced edema, indicating the efficacy of this treatment.