Involvement of mitochondria in myasthenia gravis complicated with dermatomyositis and rheumatoid arthritis: a case report

Shichijo, Kana; Mitsui, Takao; Kunishige, Makoto; Kuroda, Yukiko; Masuda, Kuniyoshi; Matsumoto, Toshio

doi:10.1007/s00401-005-0990-z

Cited by 19 publications

(7 citation statements)

References 14 publications

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“…Indeed, we found that the clinical features in patients with AMAs showed great variability. Firstly, although AMAs only occurred in adult IIM patients, the age at onset ranged from 23 years to 86 years and the mean age at onset ranged from the fourth decade to the seventh decade [3][4][5]7,8,[20][21][22][23][24][25][26] . Besides, the average disease duration varied from 10 months (in our study) to 6.5 years, and the prevalence of chronic disease duration varied from approximately 30% (in our study) to 85% [3,5,6] .…”

Section: Discussionmentioning

confidence: 99%

Idiopathic inflammatory myopathies with anti-mitochondrial antibodies: Clinical features and treatment outcomes in a Chinese cohort

Hou

Liu

Luo

et al. 2019

Neuromuscular Disorders

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Anti-mitochondrial antibodies, the hallmark of primary biliary cirrhosis, have been detected in many patients with idiopathic inflammatory myopathies and these anti-mitochondrial-antibody-associated idiopathic inflammatory myopathies frequently show unique characteristics. We detected anti-mitochondrial antibodies in Chinese idiopathic inflammatory myopathy and summarized the clinical findings of these antimitochondrial-antibody-positive patients. Of 136 patients, seven (5.15%) were found to be anti-mitochondrial-antibody-positive. Primary biliary cirrhosis was present in 2 of these 7 patients, chronic disease duration in 2 patients and asymmetrical muscle weakness in 4 patients. The mean disease course was 8.58 months, and the mean creatine kinase level was 2256.53 U/L. Myositis-specific antibodies were found in 3 patients. According to clinical features and muscle histopathological findings, 3 patients were classified as dermatomyositis, 2 as possible polymyositis and 2 as necrotizing autoimmune myopathy. Of the 6 anti-mitochondrial-antibody-positive patients receiving follow-ups of 12-83 months, they all showed marked clinical improvement. Our study indicates that anti-mitochondrial antibodies are relatively rare in Chinese idiopathic inflammatory myopathy patients. These patients generally show various clinical features and have favorable treatment outcomes. Anti-mitochondrial antibody testing may be helpful to confirm the diagnosis of idiopathic inflammatory myopathy, especially in patients with atypical manifestations.

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Section: Discussionmentioning

confidence: 99%

Idiopathic inflammatory myopathies with anti-mitochondrial antibodies: Clinical features and treatment outcomes in a Chinese cohort

Hou

Liu

Luo

et al. 2019

Neuromuscular Disorders

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“…The muscle tissue of patients with MG display abnormal mitochondrial morphology and function, and the energy levels are often significantly lower than normal ( 44 , 45 ). Similarly, abnormally shaped and structured mitochondria on muscle biopsy, ragged-red fibers, loss of mitochondrial respiratory chain complexes-1 and muscle aerobic dysfunction were observed in MG ( 46 , 47 ). These previous findings suggest the presence of mitochondrial abnormalities in neuromuscular diseases, such as MG, which may lead to muscular weakness.…”

Section: Mitochondrial Dysfunction Disordersmentioning

confidence: 80%

The mitochondrial biogenesis signaling pathway is a potential therapeutic target for myasthenia gravis via energy metabolism (Review)

Song

et al. 2021

Exp Ther Med

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Myasthenia gravis (MG) is an autoantibody-mediated autoimmune disease that is characterized by muscle weakness and fatigue. Traditional treatments for MG target the neuromuscular junction (NMJ) or the immune system. However, the efficacy of such treatments is limited, and novel therapeutic options for MG are urgently required. In the current review, a new therapeutic strategy is proposed based on the mitochondrial biogenesis and energy metabolism pathway, as stimulating mitochondrial biogenesis and the energy metabolism might alleviate myasthenia gravis. A number of cellular sensors of the energy metabolism were investigated, including AMP-activated protein kinase (AMPK) and sirtuin 1 (SIRT1). AMPK and SIRT1 are sensors that regulate cellular energy homeostasis and maintain energy metabolism by balancing anabolism and catabolism. Peroxisome proliferator-activated receptor γ coactivator 1α and its downstream transcription factors nuclear respiratory factors 1, nuclear respiratory factors 2, and transcription factor A are key sensors of mitochondrial biogenesis, which can restore mitochondrial DNA and produce new mitochondria. These processes help to control muscle contraction and relieve the symptoms of MG, including muscle weakness caused by dysfunctional NMJ transmission. Therefore, the present review provides evidence for the therapeutic potential of targeting mitochondrial biogenesis for the treatment of MG.

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“…MG and IMs are both acquired diseases caused by autoimmune responses against neuromuscular junctions and muscle tissues, respectively. Although both 2 diseases are rare, there have been cases of MG and IM co-occurrence, and it is increasingly being recognized that the comorbidity of MG and IMs could be related to thymoma as a paraneoplastic phenomenon (62)(63)(64)(65)(66)(67)(68).…”

Section: Inflammatory Myopathymentioning

confidence: 99%

Clinical features of myasthenia gravis with neurological and systemic autoimmune diseases

Zhu,

Wang,

Hao

et al. 2023

Front. Immunol.

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Multiple reports on the co-existence of autoimmune diseases and myasthenia gravis (MG) have raised considerable concern. Therefore, we reviewed autoimmune diseases in MG to explore their clinical presentations and determine whether the presence of autoimmune diseases affects the disease severity and treatment strategies for MG. We reviewed all the major immune-mediated coexisting autoimmune conditions associated with MG. PubMed, Embase and Web of Science were searched for relevant studies from their inception to January 2023. There is a higher frequency of concomitant autoimmune diseases in patients with MG than in the general population with a marked risk in women. Most autoimmune comorbidities are linked to AChR-MG; however, there are few reports of MuSK-MG. Thyroid disorders, systemic lupus erythematosus, and vitiligo are the most common system autoimmune diseases associated with MG. In addition, MG can coexist with neurological autoimmune diseases, such as neuromyelitis optica (NMO), inflammatory myopathy (IM), multiple sclerosis (MS), and autoimmune encephalitis (AE), with NMO being the most common. Autoimmune diseases appear to develop more often in early-onset MG (EOMG). MS coexists more commonly with EOMG, while IM coexists with LOMG. In addition, MG complicated by autoimmune diseases tends to have mild clinical manifestations, and the coexistence of autoimmune diseases does not influence the clinical course of MG. The clinical course of neurological autoimmune diseases is typically severe. Autoimmune diseases occur most often after MG or as a combined abnormality; therefore, timely thymectomy followed by immunotherapy could be effective. In addition, thymoma-associated AChR MG is associated with an increased risk of AE and IM, whereas NMO and MS are associated with thymic hyperplasia. The co-occurrence of MG and autoimmune diseases could be attributed to similar immunological mechanisms with different targets and common genetic factor predisposition. This review provides evidence of the association between MG and several comorbid autoimmune diseases.

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Involvement of mitochondria in myasthenia gravis complicated with dermatomyositis and rheumatoid arthritis: a case report

Cited by 19 publications

References 14 publications

Idiopathic inflammatory myopathies with anti-mitochondrial antibodies: Clinical features and treatment outcomes in a Chinese cohort

Idiopathic inflammatory myopathies with anti-mitochondrial antibodies: Clinical features and treatment outcomes in a Chinese cohort

The mitochondrial biogenesis signaling pathway is a potential therapeutic target for myasthenia gravis via energy metabolism (Review)

Clinical features of myasthenia gravis with neurological and systemic autoimmune diseases

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