The clinical and histopathological features of idiopathic inflammatory myopathies with asymmetric muscle involvement

Liu, Meirong; Hou, Ying; Dai, Tao; Lv, Jima; Li, Wei; Zhao, Yuying; Fang, Qi; Yan, Chuanzhu

doi:10.1016/j.jocn.2019.04.002

Cited by 5 publications

(2 citation statements)

References 41 publications

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“…IMNM has been characterized by subacute onset of symmetrical proximal muscle weakness, elevated CPK levels, and myopathic findings of electromyography [3,6]. In a recent clinical study [7], 134 patients with inflammatory myopathy were reviewed, and only 13 patients (9.2%) with asymmetric muscle involvement were identified, which indicated that the condition is rare. The condition primarily presented with dermatomyositis and presented as IMNM in four patients.…”

Section: Discussionmentioning

confidence: 99%

Prominent Asymmetric Muscle Weakness and Atrophy in Seronegative Immune-Mediated Necrotizing Myopathy

et al. 2021

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Immune-mediated necrotizing myopathy, a new subgroup of inflammatory myopathies, usually begins with subacute onset of symmetrical proximal muscle weakness. A 35-year-old male presented with severe asymmetric iliopsoas atrophy and low back pain with a previous history of left lower extremity weakness. Although his first left lower extremity weakness occurred 12 years ago, he did not receive a clear diagnosis. Magnetic resonance imaging of both thigh muscles showed muscle edema and contrast enhancement in patch patterns, and the left buttock and thigh muscles were more atrophied compared to the right side. Serum creatine kinase levels were elevated, and serologic testings were all negative. Genetic testing using a targeted gene-sequencing panel for neuromuscular disease including myopathy identified no pathogenic variants. Muscle biopsy on the right vastus lateralis showed scattered myofiber necrosis with phagocytosis and an absence of prominent inflammatory cells, consistent with seronegative necrotizing myopathy. Thus, unusual asymmetric muscle weakness and atrophy can be a manifestation of inflammatory myopathy.

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Section: Discussionmentioning

confidence: 99%

Prominent Asymmetric Muscle Weakness and Atrophy in Seronegative Immune-Mediated Necrotizing Myopathy

et al. 2021

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“…In regard to clinical assessments, “chronic disease duration” was defined as a duration over 12 months from the disease onset to first examination 19 . Muscle strength was evaluated by the ordinal six‐point (0–5) manual muscle testing (MMT) scale; asymmetric muscle weakness was defined as no less than 1 grade measured by MMT between two sides of the same muscle group or the same joint activity 20 . Treatment outcomes were graded as follows: no improvement, mild improvement (1 grade improvement in at least one muscle group, persistently requiring assistance in daily activities), moderate improvement (>1 grade in multiple muscle groups, occasionally requiring assistance in daily activities), marked improvement (only mild weakness without functional impairment), or return to baseline (no symptoms or signs of muscle weakness); a favorable outcome was defined as marked improvement or return to baseline 21 …”

Section: Methodsmentioning

confidence: 99%

Juvenile idiopathic inflammatory myopathies with anti‐3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase antibodies in a Chinese cohort

et al. 2021

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Aims To characterize the clinical and histopathological characteristics and treatment outcomes of juvenile idiopathic inflammatory myopathies (JIIMs) with anti‐3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase (HMGCR) antibodies in a Chinese cohort. Methods We detected anti‐HMGCR antibodies in a series of Chinese JIIM by ELISA and indirect immunofluorescence assay on HEK293 cells, and summarized the clinical findings of these anti‐HMGCR antibody‐positive patients. Results Of 32 JIIM patients, 5 (15.63%) were found to be anti‐HMGCR antibody‐positive. The disease duration was 1.20 ± 0.45 months. Statin exposure was not found. Four patients had skin lesions, while typical pathological features of dermatomyositis such as perifascicular atrophy or myxovirus resistance protein A expression were not found. The mean creatine kinase level was 16771.60 U/L. Among the four patients who received long‐term (10.46 ± 1.42 years) follow‐up, three exhibited favorable outcomes with prednisone and additional immunosuppressants. Conclusions Our study indicates that anti‐HMGCR antibodies may not be rare in Chinese JIIM. These anti‐HMGCR‐positive JIIMs were characterized by acute onset, substantially elevated creatine kinase level, and skin lesions without perifascicular changes in muscle pathology. The treatment outcome is generally favorable with the combination of steroid and immunosuppressant.

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Anti-HMGCR myopathy overlaps with dermatomyositis-like rash: a distinct subtype of idiopathic inflammatory myopathy

et al. 2021

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The clinical and histopathological features of idiopathic inflammatory myopathies with asymmetric muscle involvement

Cited by 5 publications

References 41 publications

Prominent Asymmetric Muscle Weakness and Atrophy in Seronegative Immune-Mediated Necrotizing Myopathy

Prominent Asymmetric Muscle Weakness and Atrophy in Seronegative Immune-Mediated Necrotizing Myopathy

Juvenile idiopathic inflammatory myopathies with anti‐3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase antibodies in a Chinese cohort

Anti-HMGCR myopathy overlaps with dermatomyositis-like rash: a distinct subtype of idiopathic inflammatory myopathy

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