Major histocompatibility complex and inflammatory cell subtype expression in inflammatory myopathies and muscular dystrophies

Nagappa, Madhu; Nalini, Atchayaram; Gayathri, Narayanappa

doi:10.4103/0028-3886.125264

Cited by 8 publications

(7 citation statements)

References 15 publications

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“…Nagappa et al, also demonstrated that, in patients with IIMs, the MHC class-I was upregulated along the membrane of all muscle fibers. 1 Some of the inflammatory cells were labeled with MHC class-I in the endomysium and perimysium, whereas sarcoplasmic staining was not identified in any muscle fibers including the regenerating and necrotic fibers. 1 …”

Section: Introductionmentioning

confidence: 88%

“…The major histocompatibility complex (MHC) class-I antigen is an integral membrane glycoprotein normally expressed in all nucleated cells. 1 In human, normal muscle tissue does not express MHC class-I and the expression is usually seen in normal endomysial capillaries. 1 MHC class-I plays an important role in both initiating and continuing the muscle damage.…”

Section: Introductionmentioning

confidence: 99%

“… 1 In human, normal muscle tissue does not express MHC class-I and the expression is usually seen in normal endomysial capillaries. 1 MHC class-I plays an important role in both initiating and continuing the muscle damage. It is triggered by a variety of factors, only a prolonged upregulation with abnormal expression of sarcolemmal proteins or predisposing genetic background in certain individuals may cause muscle inflammation.…”

Section: Introductionmentioning

confidence: 99%

“…It is triggered by a variety of factors, only a prolonged upregulation with abnormal expression of sarcolemmal proteins or predisposing genetic background in certain individuals may cause muscle inflammation. 1 Recent studies suggested that the lack of MHC class-I expression in normal muscle may play an important role in protecting striated muscle from apoptosis. The exact cause of increased MHC class-I expression and its specific role in the pathogenesis of different myopathic diseases, particular myositis, remains unidentified.…”

Section: Introductionmentioning

confidence: 99%

“…Since the last 30 years, MHC class-I expression has been detected in different variants of idiopathic inflammatory myopathies (IIMs). 1 , 3 , 4 Many Pathologists or Neurologists used it as a sensitive marker to distinguish IIMs from non-IIMs. van der Pas et al, evaluated 208 muscle biopsy samples, stained with MHC class-I antigen, and they found that there was no MHC class-I expression in metabolic, congenital, and neurogenic disorders as well as healthy controls.…”

Section: Introductionmentioning

confidence: 99%

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The Assessment of Major Histocompatibility Complex (MHC) Class-I Expression in Different Neuromuscular Diseases

Kurdi¹,

Alshareef²,

Bamaga³

et al. 2021

DNND

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Background Major histocompatibility complex (MHC) class-1 antigen is a glycoprotein expressed in all nucleated cells. The aim of this study was to assess MHC class-I expression in different neuromuscular diseases. Methods The authors reviewed the data of 54 patients with neuromuscular diseases. Anti MHC class-I antibody was performed on the frozen muscle tissues using immunohistochemistry. MHC class-I was scored based on its expression on muscle fibers (0: normal, 1: expression <5 fibers, 2: expression in 5–10 fibers, 3: expression in >10 fibers). The pattern was only assessed in cases with MHC class-I scored 3 as: (1: Sarcocapillary, 2: Sarcocapillary and necrotic fibers, 3: Perifascicular). The relationship between MHC class-I expression and neuromuscular diseases was statistically analyzed. Results The mean age of the patients was 39.1 ± 18.5 years. Around 50% of patients showed normal CK levels and 5% of the cases showed elevated CK levels. There was a significance difference in MHC class-I expression between cases with normal and elevated CK levels when MHC class-I score was 3 (p= 0.020). There was a significant difference in MHC class-I expression among different neuromuscular diseases (p<0.001). All cases with idiopathic inflammatory myopathies (IIMs) have expressed MHC class-I in more than 10 fibers. MHC class-I was expressed in 15 cases of non-IIMs. Conclusion MHC class-I cannot be solely used as a biomarker to distinguish IIMs from non-IIMs. The presence of MHC class-I molecules in non-IIMs might be related to immunoproteasomes mechanism. Further studies, with different muscle proteins expression and genomic sequencing, must be conducted to understand the role of MHC Class-I in neuromuscular diseases.

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Section: Introductionmentioning

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

The Assessment of Major Histocompatibility Complex (MHC) Class-I Expression in Different Neuromuscular Diseases

Kurdi¹,

Alshareef²,

Bamaga³

et al. 2021

DNND

View full text Add to dashboard Cite

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Immunobiology of Inherited Muscular Dystrophies

Tidball

Welc

Wehling‐Henricks

2018

Comprehensive Physiology

116

126

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The immune response to acute muscle damage is important for normal repair. However, in chronic diseases such as many muscular dystrophies, the immune response can amplify pathology and play a major role in determining disease severity. Muscular dystrophies are inheritable diseases that vary tremendously in severity, but share the progressive loss of muscle mass and function that can be debilitating and lethal. Mutations in diverse genes cause muscular dystrophy, including genes that encode proteins that maintain membrane strength, participate in membrane repair, or are components of the extracellular matrix or the nuclear envelope. In this article, we explore the hypothesis that an important feature of many muscular dystrophies is an immune response adapted to acute, infrequent muscle damage that is misapplied in the context of chronic injury. We discuss the involvement of the immune system in the most common muscular dystrophy, Duchenne muscular dystrophy, and show that the immune system influences muscle death and fibrosis as disease progresses. We then present information on immune cell function in other muscular dystrophies and show that for many muscular dystrophies, release of cytosolic proteins into the extracellular space may provide an initial signal, leading to an immune response that is typically dominated by macrophages, neutrophils, helper T-lymphocytes, and cytotoxic T-lymphocytes. Although those features are similar in many muscular dystrophies, each muscular dystrophy shows distinguishing features in the magnitude and type of inflammatory response. These differences indicate that there are disease-specific immunomodulatory molecules that determine response to muscle cell damage caused by diverse genetic mutations. © 2018 American Physiological Society. Compr Physiol 8:1313-1356, 2018.

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The composition of cellular infiltrates in anti‐HMG‐CoA reductase‐associated myopathy

et al. 2015

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Objective To characterize cellular infiltrates in muscle biopsies from patients with anti-3-hydroxy-3-methyl-gulatryl-CoA reductase (HMGCR)-associated myopathy. Methods Biopsies from 18 anti-HMGCR myopathy and 7 control dermatomyositis patients were analyzed. Results CD4+ and CD8+ T-cells were scattered within the endomysium in 50% of anti-HMGCR biopsies. All anti-HMGCR biopsies included increased endomysial and/or perivascular CD163+ M2 macrophages; CD11c+ M1 macrophages were present in 18.8%. CD123+ plasmacytoid dendritic (PD) cells were observed within the endomysium and perivascular spaces in 62.5% of anti-HMGCR biopsies. Membrane attack complex was deposited on endothelial cells in 50% and on the sarcolemma of non-necrotic muscle fibers in 85.7% of anti-HMGCR cases. Major histocompatibility complex class I antigen was up-regulated in 87.5% of the anti-HMGCR cases. Conclusion In addition to necrosis, scattered CD4+, CD8+, and PD cells are characteristic of anti-HMGCR myopathy. Predominant M2 polarization suggests infiltrating macrophages are more likely to be involved with tissue repair than destruction.

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Major histocompatibility complex and inflammatory cell subtype expression in inflammatory myopathies and muscular dystrophies

Abstract: The pattern of MHC-I and II expression appeared to be similar in both inflammatory myopathies as well as in muscular dystrophies with inflammation and hence differentiating them on MHC - I expression was difficult.

Cited by 8 publications

References 15 publications

The Assessment of Major Histocompatibility Complex (MHC) Class-I Expression in Different Neuromuscular Diseases

The Assessment of Major Histocompatibility Complex (MHC) Class-I Expression in Different Neuromuscular Diseases

Immunobiology of Inherited Muscular Dystrophies

The composition of cellular infiltrates in anti‐HMG‐CoA reductase‐associated myopathy

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