Analysis of immunoglobulin secretion by lymph organs with myasthenia gravis

Yoshikawa, Hiroaki; Satoh, Katsuaki; Yasukawa, Yoshihiro; Yamada, Masahito

doi:10.1034/j.1600-0404.2001.00209.x

Cited by 13 publications

(10 citation statements)

References 16 publications

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“…Regarding the four types of treatment (based on the initial treatment program), 39 patients were classified to the single pyridinium bromide neostigmine group, 26 patients to the transitional reduction of oral prednisone group, 7 patients to the pyridinium bromide neostigmine in combination with reduction of prednisone group and 61 patients to the methylprednisolone pulse therapy group. The mean treatment onset time was 13 ( 10 – 17 ), 11 ( 4 – 15 ), 8 ( 1 – 12 ) and 2 ( 1 – 6 ) days, respectively. The mean duration of the maximum effect was 74 (23–212), 52 (19–174), 48 (7–125) and 18 (3–54) days, respectively, with no significant differences among the subtypes.…”

Section: Resultsmentioning

confidence: 99%

Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Yang

Xiong

2015

Experimental and Therapeutic Medicine

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This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed and prospectively monitored. Of the 135 MG patients, 85.2% had type I (ocular type), with only 4.2% progressing to systemic MG; 13.4% had type II (general type); and 1.5% had type III (fulminating type). Relapse occurred in 46.1% of the 102 patients that were followed up. The positive rate for the primary acetylcholine receptor antibody was 40.19%, without significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by electromyography was 37.97%. Decreased expression of CD4+, CD8+, or CD3+ was present in 71% of the patients. Thymic hyperplasia was present in 5.93% of the patients, while 1.48% had thymoma. Steroid treatment was effective in the majority of the patients. Ocular type MG was common in this cohort of patients. The incidence and mortality of myasthenia crisis were low, the presence of concurrent thymoma was rare and only a limited number of children developed neurological sequelae.

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Section: Resultsmentioning

confidence: 99%

Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Yang

Xiong

2015

Experimental and Therapeutic Medicine

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“…Isolated B cells from thymic hyperplasia spontaneously produce anti‐AChR antibodies 4 . In the periphery, total Ig production is higher in MG patients than in age‐matched healthy control individuals, 24 suggesting that the increase in Ig synthesis is not restricted to anti‐AChR antibodies. In this context, antibodies against several autoantigens other than AChR have been detected in MG patients, in particular muscle‐cell antigens, such as titin or ryanodine receptor 25 .…”

Section: Th Subsets and Activation Markers In Mg Immune Cellsmentioning

confidence: 99%

Defects of immunoregulatory mechanisms in myasthenia gravis: role of IL‐17

Gradolatto

Nazzal

Foti

et al. 2012

Annals of the New York Academy of Sciences

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Deficient immunoregulation is consistently observed in autoimmune diseases. Here, we summarize the abnormalities of the T cell response in autoimmune myasthenia gravis (MG) by focusing on activation markers, inflammatory features, and imbalance between the different T cell subsets, including Th17 and regulatory T cells (T(reg) cells). In the thymus from MG patients, T(reg) cell numbers are normal while their suppressive function is severely defective, and this defect could not be explained by contaminating effector CD127(low) T cells. A transcriptomic analysis of T(reg) cell and conventional T cell (T(conv) ; CD4(+) CD25(-) cells) subsets pointed out an upregulation of Th17-related genes in MG cells. Together with our previous findings of an inflammatory signature in the MG thymus and an overproduction of IL-1 and IL-6 by MG thymic epithelial cells (TEC), these data strongly suggest that T cell functions are profoundly altered in the thymic pathological environment. In this short review we discuss the mechanisms of chronic inflammation linked to the pathophysiology of MG disease.

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“…Thus, these data provide evidence for persistent clonally expanded B helper CD4+ T cell populations in the blood of MG patients. 6 In the periphery, total Ig production is higher in the blood of MG patients than in age-matched healthy control (HC) individuals, 7 indicating an active inflammatory state. The detection of MG-specific abs helps the diagnosis, along with clinical fatigue tests and neurophysiological assessment of neuromuscular transmission failure.…”

Section: Introductionmentioning

confidence: 99%

Circulating miRNAs in myasthenia gravis: miR‐150‐5p as a new potential biomarker

Punga

Panse

Andersson³

et al. 2013

Ann Clin Transl Neurol

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ObjectiveMyasthenia gravis (MG) is a chronic autoimmune disorder where autoantibodies target the nicotinic acetylcholine receptors (AChR+) in about 85% of cases, in which the thymus is considered to play a pathogenic role. As there are no reliable biomarkers to monitor disease status in MG, we analyzed circulating miRNAs in sera of MG patients to find disease-specific miRNAs.MethodsOverall, 168 miRNAs were analyzed in serum samples from four AChR+ MG patients and four healthy controls using Exiqon Focus miRNA polymerase chain reaction (PCR) Panel I + II. Specific accumulation pattern of 13 miRNAs from the discovery set was subsequently investigated in the sera of 16 AChR+ MG patients and 16 healthy controls. All patients were without immunosuppressive treatment. Selected specific miRNAs were further analyzed in the serum of nine MG patients before and after thymectomy to assess the effect of thymus removal on the accumulation of the candidate miRNAs in patient sera.ResultsThree miRNAs were specifically dysregulated in AChR+ MG patient sera samples. Hsa-miR150-5p, which induces T-cell differentiation, as well as hsa-miR21-5p, a regulator of Th1 versus Th2 cell responses, were specifically elevated in MG sera. Additionally, hsa-miR27a-3p, involved in natural killer (NK) cell cytotoxicity, was decreased in MG. Hsa-miR150-5p levels had the highest association with MG and were significantly reduced after thymus removal in correlation with disease improvement.InterpretationWe propose that the validated miRNAs: hsa-miR150-5p, hsa-miR21-5p, and hsa-miR27a-3p can serve as novel serum biomarkers in AChR+ MG. Hsa-miR-150-5p could be a helpful marker to monitor disease severity.

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Analysis of immunoglobulin secretion by lymph organs with myasthenia gravis

Abstract: PBMC is the most efficient site at producing AChRAb, even in patients of short duration (equal to, or less than, 2 months). Monitoring AChRAb secretion by PBMC is useful to estimate the autoimmune activity of MG.

Cited by 13 publications

References 16 publications

Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Defects of immunoregulatory mechanisms in myasthenia gravis: role of IL‐17

Circulating miRNAs in myasthenia gravis: miR‐150‐5p as a new potential biomarker

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