Inflammatory myopathy with myasthenia gravis

Uchio, Naohiro; Taira, Kenichiro; Ikenaga, Chiseko; Kadoya, Masato; Unuma, Atsushi; Yoshida, Kenji; Nakatani-Enomoto, Setsu; Hatanaka, Y.; Sakurai, Yasuhisa; Shiio, Yasushi; Kaida, Kenichi; Kubota, Akatsuki; Toda, Tatsushi; Shimizu, Jun

doi:10.1212/nxi.0000000000000535

Cited by 32 publications

(9 citation statements)

References 42 publications

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“…On the hand, concomitant myositis or myocarditis is rarely observed in idiopathic MG and is usually reported in cases of thymoma-associated MG [31,32]. Interestingly, we detected anti-titin antibodies in three out of four patients with MG/myositis/myocarditis (75%), confirming previous reports [33,34].…”

Section: Discussionsupporting

confidence: 89%

“…The co-occurrence of myocarditis [ 28 ] and the elevation of serum troponin [ 29 ] have been identified as predictors of poor prognosis in patients with ir-MG/myositis and IM3OS is characterized by a high mortality (up to 60% of cases) [ 30 ], which can occur due to both cardiac (i.e., arrythmia) and neuromuscular (i.e., respiratory failure) involvement. On the other hand, concomitant myositis or myocarditis is rarely observed in idiopathic MG and is usually reported in cases of thymoma-associated MG [ 31 , 32 ]. Interestingly, we detected anti-titin antibodies in three out of four patients with MG/myositis/myocarditis (75%), confirming previous reports [ 33 , 34 ].…”

Section: Discussionmentioning

confidence: 99%

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Peripheral nervous system adverse events associated with immune checkpoint inhibitors

et al. 2023

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Background Immune checkpoint inhibitors (ICIs) represent an effective cancer immunotherapy yet are associated with immune-related adverse events (irAEs). The aim of this study was to characterize irAEs involving the peripheral nervous system (PNS-irAEs) in a real-world cohort of ICI-treated patients. Methods Cancer patients treated with ICIs between January 2014 and March 2022 were included. Patients with PNS-irAEs were identified and divided into two groups: (1) cranial/peripheral neuropathies and (2) myasthenia gravis (MG) and/or myositis. Clinical characteristics and outcomes, measured with the modified Rankin Scale (mRS), were compared among the two groups. Results Among 920 ICI-treated patients, 20 patients (2.17%) developed a PNS-irAEs. The median latency from ICI exposure was 8.8 weeks and the median time from onset to clinical nadir was 3.5 weeks. Eleven patients developed a neuropathy: polyneuropathy (n = 4), cranial neuropathy (n = 3), small-fiber neuropathy (n = 3), brachial plexopathy (n = 1). Nine patients presented MG and/or myositis: concomitant MG and myositis (n = 6), isolated myositis (n = 2), exacerbation of MG (n = 1). Immunosuppressive treatment and/or ICI withdrawal determined a significant clinical improvement, expressed by a mRS reduction, in the neuropathy group (p = 0.004), but not in the MG/myositis group (p = 0.11). Overall, death due to irAEs occurred in four patients (20%), all with MG/myositis. Compared to patients with neuropathies, those with MG/myositis had a shorter latency onset (p = 0.036), developed more frequently concomitant non-neurologic irAEs (p = 0.028) and showed a higher mortality rate (p = 0.026). Conclusions In our large cohort of ICI-treated patients, 2.17% developed PNS-irAEs. Compared to ir-neuropathies, ir-MG/myositis tend to occur earlier from ICI exposure and present a worse response to treatment and a higher mortality.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Peripheral nervous system adverse events associated with immune checkpoint inhibitors

et al. 2023

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“…Suzuki et al demonstrated that the prevalence of inflammatory myopathies in a cohort of 924 MG patients was 0.9% (8/924) [ 16 ]. Similar co-existing diagnoses were reported in an analysis of 970 confirmed IIM patients, where Uchio and colleagues found that the prevalence of concomitant MG was 1.03% (10/970) [ 40 ]. The clinicopathologic features of these 10 patients were comparable to those in our literature review.…”

Section: Discussionsupporting

confidence: 77%

Giant cell myositis associated with concurrent myasthenia gravis: a case-based review of the literature

et al. 2021

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The term “giant cell myositis” has been used to refer to muscle diseases characterized histologically by multinucleated giant cells. Myasthenia gravis is an autoimmune neuromuscular junction disorder. The rare concurrence of giant cell myositis with myasthenia gravis has been reported; however, the clinical and histological features have varied widely. Here, we present such a case and a review of the literature. An 82-year-old woman admitted for subacute, progressive, proximal muscle weakness developed acute-onset dysphagia, dysphonia, and respiratory distress 5 days after admission. Laboratory findings were positive for acetylcholine receptor binding antibodies and striational muscle antibodies against titin. Muscle biopsy demonstrated widespread muscle fiber necrosis with multinucleated giant cells, consistent with giant cell myositis. She died despite treatment with pulse methylprednisolone and plasma exchange. A literature review of the PubMed and Scopus databases from 1944 to 2020 identified 15 additional cases of these co-existing diagnoses. We found that giant cell myositis with myasthenia gravis primarily affects female patients, is typically diagnosed in the 6–7th decades, and is characterized by the presence of thymoma. Muscle histology predominantly shows giant cell infiltrate without granulomas. The onset of myasthenia gravis symptoms may precede, follow, or coincide with symptoms of myositis. Treatment with thymectomy, anticholinesterase inhibitors, or immunosuppressive therapy may lead to favorable clinical outcomes.

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“…In cases where the initial diagnosis was MG, patients presented with typical MG symptoms, followed by myopathy, with a predominant association with polymyositis [11,12]. Thymoma has been associated with rhabdomyolysis-like features, respiratory failure, and dropped head; however, cardiac involvement and ocular symptoms are more commonly observed [13]. Nonetheless, the association between MG and IIM without thymic pathology occurs in 45% of cases [14,15].…”

Section: Discussionmentioning

confidence: 99%

Myasthenia Gravis with Idiopathic Inflammatory Myopathy and Sjögren Syndrome, A Triad of Immune-Mediated Diseases

Cobilt-Catana¹

2023

J Psy Neurol.

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This report illustrates a 73-year-old woman with Sjögren's syndrome and idiopathic inflammatory myopathy with elevated creatine kinase that subsequently deteriorated with an increased dose of steroids, resulting in a myasthenic crisis requiring hospitalization in the intensive care unit; the patient responded successfully to treatment with intravenous immunoglobulin. The patient showed moderate elevation of creatine kinase at the initial diagnosis, with positive anti-Ro/SSA antibodies. Schirmer’s test, anti-Ro52 and anti-Ku antibodies, electromyography, and muscle biopsy were performed to confirm inflammatory myopathy. During the observation in the intensive care unit, creatine kinase increased to 1520 U/L, and a repetitive stimulation test and total anti-acetylcholine receptor antibodies supported the diagnosis of myasthenia gravis. The patient received intravenous immunoglobulin, evolving with clinical improvement. This case is being reported to illustrate the chronic clinical course of a patient with inflammatory myopathy who deteriorated following steroid adjustment, ultimately presenting with myasthenic crisis.

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Inflammatory myopathy with myasthenia gravis

Cited by 32 publications

References 42 publications

Peripheral nervous system adverse events associated with immune checkpoint inhibitors

Peripheral nervous system adverse events associated with immune checkpoint inhibitors

Giant cell myositis associated with concurrent myasthenia gravis: a case-based review of the literature

Myasthenia Gravis with Idiopathic Inflammatory Myopathy and Sjögren Syndrome, A Triad of Immune-Mediated Diseases

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