Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies

Farini, Andrea; Villa, Chiara; Tripodi, Luana; Legato, Mariella; Torrente, Yvan

doi:10.3389/fimmu.2021.666879

Cited by 8 publications

(4 citation statements)

References 109 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Many cell types have been hypothesized to be involved in muscle disease, such as satellite cells in Duchenne muscular dystrophy (Chang et al, 2016), fiboradipogenic progenitors in muscular dystrophies, other myopathies, and aging (Molina et al, 2021;Theret et al, 2021), and various immune populations as both integral parts of injury response and pathology when dysregulated (Chen & Shan, 2019;Farini et al, 2021;Venalis & Lundberg, 2014). However, we believe that examining myogenic cells is also crucial to gain a full understanding of the muscle environment.…”

Section: Discussionmentioning

confidence: 97%

A protocol for single nucleus RNA-seq from frozen skeletal muscle

et al. 2023

View full text Add to dashboard Cite

Single-cell technologies are a method of choice to obtain vast amounts of cell-specific transcriptional information under physiological and diseased states. Myogenic cells are resistant to single-cell RNA sequencing because of their large, multinucleated nature. Here, we report a novel, reliable, and cost-effective method to analyze frozen human skeletal muscle by single-nucleus RNA sequencing. This method yields all expected cell types for human skeletal muscle and works on tissue frozen for long periods of time and with significant pathological changes. Our method is ideal for studying banked samples with the intention of studying human muscle disease.

show abstract

Section: Discussionmentioning

confidence: 97%

A protocol for single nucleus RNA-seq from frozen skeletal muscle

et al. 2023

View full text Add to dashboard Cite

show abstract

“…In addition to the primary cause, DMD is associated with high levels of oxidative stress and inflammation, which actively exacerbate the dystrophic phenotype. Although oxidative stress and inflammation are crucial for tissue regeneration after initial damage, their constant presence in skeletal muscles leads to further necrosis, injury, and accumulation of fibrosis [ 122 , 123 , 124 , 125 , 126 , 127 ]. Sustained and excessive infiltration of macrophages, as described in dystrophic muscles, disrupts redox homeostasis and causes muscle damage [ 128 ].…”

Section: Skeletal Muscle Regeneration In Pathological Conditionsmentioning

confidence: 99%

“…In particular, the low chronic inflammation during aging induces SCs to move toward a senescence phenotype that increases the secretion of inflammatory cytokines such as TNF-α and IL-6 [ 95 , 96 ]. On the other hand, skeletal muscle dystrophy is characterized by an increased number of inflammatory cells, while cancer cachexia does not affect the cellular component but induces a significant enhancement of secreted cytokines, aggravating the pathological phenotype by increasing autoinflammation and fibrotic tissue deposition [ 123 , 124 , 175 , 176 ]. Another factor secreted by inflammatory cells that plays a central role in altering muscle regeneration is TGF-β.…”

Section: Summary and Future Prospectivementioning

confidence: 99%

Regulation of Satellite Cells Functions during Skeletal Muscle Regeneration: A Critical Step in Physiological and Pathological Conditions

Careccia,

Mangiavini,

Cirillo

2023

IJMS

View full text Add to dashboard Cite

Skeletal muscle regeneration is a complex process involving the generation of new myofibers after trauma, competitive physical activity, or disease. In this context, adult skeletal muscle stem cells, also known as satellite cells (SCs), play a crucial role in regulating muscle tissue homeostasis and activating regeneration. Alterations in their number or function have been associated with various pathological conditions. The main factors involved in the dysregulation of SCs’ activity are inflammation, oxidative stress, and fibrosis. This review critically summarizes the current knowledge on the role of SCs in skeletal muscle regeneration. It examines the changes in the activity of SCs in three of the most common and severe muscle disorders: sarcopenia, muscular dystrophy, and cancer cachexia. Understanding the molecular mechanisms involved in their dysregulations is essential for improving current treatments, such as exercise, and developing personalized approaches to reactivate SCs.

show abstract

“…In recent years, an additional role for Ig therapy in the treatment of various inflammatory and immune-mediated diseases has been proposed (14,15). Consequently, although the related mechanisms of action are complex and only partially understood, the use of SCIg treatment has expanded from immunodeficiencies to autoimmune diseases, as polymyositis (PM) and dermatomyositis (DM) (16)(17)(18)(19).…”

Section: Introductionmentioning

confidence: 99%

Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients

et al. 2022

View full text Add to dashboard Cite

BackgroundImmunoglobulin (Ig) replacement therapy represents a life-saving treatment in primary antibody deficiencies. The introduction of subcutaneous Ig (SCIg) administration brings a major improvement in quality of life for patients, compared to the traditional intravenous administration. In recent years, an additional role has been proposed for Ig therapy for various inflammatory and immune-mediated diseases. Consequently, the use of SCIg has expanded from immunodeficiencies to immune-mediated diseases, such as polymyositis (PM) and dermatomyositis (DM). Given the rarity of these conditions, it is still difficult to evaluate the real impact of SCIg treatment on PM and DM, and additional data are constantly required on this topic, particularly for long-term treatments in real-life settings.AimThis study aimed to increase the knowledge about the anti-inflammatory and immunomodulatory effects of SCIg treatment for myositis. To this aim, a long-term evaluation of the effectiveness of 20% human SCIg treatment (20% SCIg, Hizentra®, CSL Behring) was carried out in patients with PM/DM in care at our Center. In addition, an evaluation of the 20% SCIg therapy in CVID patients was provided. This analysis, beside adding knowledge about the use of SCIg therapy in this real-life setting, was intended as a term of comparison, regarding the safety profile.ResultsResults support the beneficial effect and tolerability of long-term 20% SCIg therapy in PM/DM patients, reporting a significant improvement in creatine kinase levels, muscle strength, skin conditions, dysphagia, disease activity (MITAX score) and disability (HAQ-DI score). None of the patients reported systemic reactions. The duration of the reported local reactions was a few hours in 80% of the patients, and all resolved spontaneously. CVID patients reported an improvement in all the considered effectiveness parameters at the end of 20% SCIg therapy. The frequency of the adverse events reported by PM/DM patients was not different from what reported in CVID patients, where the use of SCIg therapy is more consolidatedConclusionsThis study suggests that 20% SCIg treatment represents a viable and safe treatment for PM/DM patients and a valid therapeutic alternative to IVIg, with important advantages for patients’ quality of life.

show abstract

Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies

Cited by 8 publications

References 109 publications

A protocol for single nucleus RNA-seq from frozen skeletal muscle

A protocol for single nucleus RNA-seq from frozen skeletal muscle

Regulation of Satellite Cells Functions during Skeletal Muscle Regeneration: A Critical Step in Physiological and Pathological Conditions

Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients

Contact Info

Product

Resources

About