Muscle involvement in myasthenia gravis: Expanding the clinical spectrum of Myasthenia-Myositis association from a large cohort of patients

Garibaldi, Matteo; Fionda, Laura; Vanoli, Fiammetta; Leonardi, Luca; Loreti, Simona; Bucci, Elisabetta; Pasquale, Antonella Di; Morino, Stefania; Vizzaccaro, Elisa; Merlonghi, Gioia; Ceccanti, Marco; Lucchini, Matteo; Mirabella, Massimiliano; Andreetta, Francesca; Pennisi, E.; Petrucci, Antonio; Salvetti, Marco; Antonini, Giovanni

doi:10.1016/j.autrev.2020.102498

Cited by 36 publications

(37 citation statements)

References 38 publications

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“…Furthermore, inflammatory myopathy is another rare AD which can coexist with MG. Garibaldi et al observed that 13 out of 441 (2.9%) MG patients developed myositis and 10/13 patients occurred simultaneously ( 26 ). So the authors recommended myositis should be considered when MG patients had the features of elevated serum CK levels or stable muscle weakness unresponsive to acetylcholinesterase inhibitors ( 26 ), in particular for MG patients with thymoma ( 27 ). As for the three patients we reported, they all had normal serum CK levels and responded well to the acetylcholinesterase inhibitors.…”

Section: Discussionmentioning

confidence: 99%

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Zhao

Liao

et al. 2020

Front. Neurol.

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Objective: The coexistence of myasthenia gravis (MG) and primary Sjögren's syndrome (pSS) is rarely reported. This study aims to describe the clinical features, treatment and outcome of MG coexisting with pSS. Materials and Methods: Herein we reported three cases with the two coexisting diseases, and also searched the PubMed, Medline databases, and China Wanfang databases for the relevant case reports written in English, Chinese, or Japanese with detailed data. Results: We reviewed a total of 17 patients with both diseases. Fifteen patients were female. The median age at onset was 48 years (range 28–78 years). MG was the initial disease in nine of 17 cases. The median interval between the onsets of the two diseases was 30 months (range 7 months to 20 years). The symptoms of MG included fatigable ptosis (64.7%), bulbar symptoms (58.8%), muscle fatigability (64.7%), diplopia (64.7%), dyspnea (23.5%), and facial paralysis (5.9%). Anti-acetylcholine receptor antibody was positive in 70.6% patients. All the patients had sicca symptoms. Manifestations of pSS also included swollen exocrine glands (23.5%), joint pain (23.5%), hair loss (11.8%), leukopenia (11.8%), recurrent oral ulcers (5.9%), Raynaud phenomenon (5.9%), and fever (5.9%). ANA positivity was present in 70.6% patients, anti-SSA positivity in 47.1%, and double positivity of anti-SSA and anti-SSB in 17.6%. There were 12 patients (70.6%) with two autoimmune diseases (pSS and MG), and five patients with more than two autoimmune diseases. Cholinesterase inhibitors were the most commonly prescribed drugs (82.4%). Seven patients received thymectomy and one patient improved after the operation. Two patients were given intravenous methylprednisolone pulse therapy, and four patients oral steroids combined with immunosuppressants initially. Intravenous immunoglobulin and plasma exchange were used in two patients, respectively, for the respiratory failure. All the patients improved following treatment except one patient who died of MG crisis due to medication withdrawal. Conclusion: The coexistence of SS with MG is quite rare. The onset of MG may occur before or after the diagnosis of SS. Co-morbidity with MG does not seem to adversely affect the course of SS. Thus, controlling the progress of MG is the critical aspect of treatment.

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Section: Discussionmentioning

confidence: 99%

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Zhao

Liao

et al. 2020

Front. Neurol.

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“…These findings in the frontalis muscle were more likely to be suggestive of myasthenia and positive antibodies against acetylcholine receptors supported our hypothesis. Pathology in MG can either be normal or can reveal muscle atrophy, or endomysial and perivascular lymphocytic infiltrates, but lacks diagnostic utility, unlike IM, where biopsy constitutes the gold standard showing, in our case, muscle fibre necrosis and pathological Human-Leukocyte-Antigen antibody staining typical of polymyositis [3]. Steroids are the mainstay of treatment in IM [1] and MG [2]; nevertheless, we do not discount that high doses at the start could have worsened weakness in our patient [2].…”

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confidence: 84%

“…Muscle involvement in MG has been barely reported [3][4][5][6], sometimes related to other autoimmune disorders [4] or immunotherapy [6]. To our knowledge, only one case with acute onset has been described [5].…”

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confidence: 94%

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Acute concomitant presentation of polymyositis and atypical myasthenia gravis: a diagnostic challenge

Natera‐Villalba

Corral‐Corral

Cabañes

et al. 2020

Euro J of Neurology

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“…Anti-Kv1.4 antibodies were first discovered by our group in 2005 using a protein immunoprecipitation assay using 35 S-labeled rhabdomyosarcoma cellular extracts. 8 The voltage-gated potassium channel consists of four transmembrane α-subunits that combine as homo-or heterotetramers.…”

Section: Autoa Nti G En Smentioning

confidence: 99%

Anti‐striational antibodies: Expanding their clinical significance

Suzuki

Nagane

Uzawa

et al. 2020

Clinical & Exp Neuroim

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Myasthenia gravis (MG) is an organ-specific autoimmune disorder generally mediated by anti-acetylcholine receptor (AChR) or, less frequently, by anti-muscle-specific tyrosine antibodies at the neuromuscular junction. 1 In the 1970s, before the discovery of anti-AChR antibodies, anti-striational antibodies (StrAbs) were known as serum immunoglobulins that react with cross-striations of skeletal muscle in MG patients. Indirect immunofluorescence of animal skeletal muscle tissue was the original method for detecting StrAbs. 2 StrAbs had been expected to be useful as biomarkers of MG; however, immunoreactivity on indirect immunofluorescence was also observed in other disorders and in normal controls. Because of the low specificity of StrAbs as biomarkers, the diagnostic utility of StrAbs had been regarded to be limited. In the 1990s, several molecules on skeletal and heart muscle proteins were identified as autoantigens of StrAbs. In particular, the

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Muscle involvement in myasthenia gravis: Expanding the clinical spectrum of Myasthenia-Myositis association from a large cohort of patients

Cited by 36 publications

References 38 publications

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review

Acute concomitant presentation of polymyositis and atypical myasthenia gravis: a diagnostic challenge

Anti‐striational antibodies: Expanding their clinical significance

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