Anti-MuSK antibodies: Correlation with myasthenia gravis severity

Bartoccioni, Emanuela; Scuderi, Flavia; Minicuci, Giacomo; Marino, Mariapaola; Ciaraffa, Francesca; Evoli, Amelia

doi:10.1212/01.wnl.0000228225.23349.5d

Cited by 134 publications

(93 citation statements)

References 11 publications

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“…Nevertheless, much fewer of the MuSK-CBA positive patients (4.6%) presented with myasthenic crises compared to the 25-48% reported for typical AChR-MG and MuSK-MG (Oh, 2009;Evoli and Padua, 2013). A correlation of antibody levels and disease severity has been suggested in previous studies of MuSK-MG patients (Bartoccioni et al, 2006). Our results could support this observation as the CBA might detect patients with lower anti-MuSK levels, which accordingly might have milder disease.…”

Section: Discussionsupporting

confidence: 75%

MuSK autoantibodies in myasthenia gravis detected by cell based assay — A multinational study

Tsonis

Zisimopoulou

Lazaridis

et al. 2015

Journal of Neuroimmunology

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Seronegative myasthenia gravis (MG) presents a serious gap in MG diagnosis and understanding. We applied a cell based assay (CBA) for the detection of muscle specific kinase (MuSK) antibodies undetectable by radioimmunoassay. We tested 633 triple-seronegative MG patients' sera from 13 countries, detecting 13% as positive. MuSK antibodies were found, at significantly lower frequencies, in 1.9% of healthy controls and 5.1% of other neuroimmune disease patients, including multiple sclerosis and neuromyelitis optica. The clinical data of the newly diagnosed MuSK-MG patients are presented. 27% of ocular seronegative patients were MuSK antibody positive. Moreover, 23% had thymic hyperplasia suggesting that thymic abnormalities are more common than believed.

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Section: Discussionsupporting

confidence: 75%

MuSK autoantibodies in myasthenia gravis detected by cell based assay — A multinational study

Tsonis

Zisimopoulou

Lazaridis

et al. 2015

Journal of Neuroimmunology

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“…There is often a remarkable response to PLEX, while the response to IVIg is often more modest [21]. Anti-MuSK antibody titers appear to correlate with severity of disease and response to immunotherapy [52,53]. In contrast, AChR antibody titers do not reliably predict disease severity in individual patients [2].…”

Section: Present Management Of Autoimmune Mgmentioning

confidence: 99%

Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

2016

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Myasthenia gravis (MG) is an autoimmune disease associated with the production of autoantibodies against 1) the skeletal muscle acetylcholine receptor; 2) muscle-specific kinase, a receptor tyrosine kinase critical for the maintenance of neuromuscular synapses; 3) low-density lipoprotein receptor-related protein 4, an important molecular binding partner for muscle-specific kinase; and 4) other muscle endplate proteins. In addition to the profile of autoantibodies, MG may be classified according the location of the affected muscles (ocular vs generalized), the age of symptom onset, and the nature of thymic pathology. Immunopathologic events leading to the production of autoantibodies differ in the various disease subtypes. Advances in our knowledge of the immunopathogenesis of the subtypes of MG will allow for directed utilization of the ever-growing repertoire of therapeutic agents that target distinct nodes in the immune pathway relevant to the initiation and maintenance of autoimmune disease. In this review, we examine the pathogenesis of MG subtypes, current treatment options, and emerging new treatments and therapeutic targets.

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“…MuSK serum autoantibody titer associates with clinical presentation (25,57) and may be predictive of relapse following rituximab treatment. We speculate that our study presents findings that may also have potential clinical utility.…”

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confidence: 99%

Autoantibody-producing plasmablasts after B cell depletion identified in muscle-specific kinase myasthenia gravis

Stathopoulos¹,

Kumar²,

Nowak³

et al. 2017

JCI Insight

137

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Anti-MuSK antibodies: Correlation with myasthenia gravis severity

Cited by 134 publications

References 11 publications

MuSK autoantibodies in myasthenia gravis detected by cell based assay — A multinational study

MuSK autoantibodies in myasthenia gravis detected by cell based assay — A multinational study

Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

Autoantibody-producing plasmablasts after B cell depletion identified in muscle-specific kinase myasthenia gravis

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