Italian recommendations for the diagnosis and treatment of myasthenia gravis

Evoli, Amelia; Antonini, Giovanni; Antozzi, Carlo; DiMuzio, Antonio; Habetswallner, Francesco; Iani, Cesare; Inghilleri, Maurizio; Liguori, Rocco; Mantegazza, Renato; Massa, Roberto; Pegoraro, Elena; Ricciardi, Rocco; Rodolico, Carmelo

doi:10.1007/s10072-019-03746-1

Cited by 48 publications

(71 citation statements)

References 72 publications

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“…Italian recommendations state that treatment is aimed at achieving satisfactory control of symptoms and minimizing the risk of adverse events. 13 The international consensus guidance focused on the Myasthenia Gravis Foundation of America Task Force Post-Intervention Status classification of MM or better. 15 with no more than grade 1 Common Terminology Criteria for Adverse Events medication side effects.…”

Section: Tre Atment G Oal Smentioning

confidence: 99%

“…10 In MuSK-MG, pyridostigmine should be used with caution because it is often ineffective and sometimes has adverse effects. Italian guidelines recommend not using pyridostigmine in MuSK-MG. 13 Japanese guidelines regard anticholinesterase drugs as supplementary agents, especially in generalized MG. 5 Most of the guidelines we consulted advise the initiation of oral steroids if treatment with pyridostigmine does not resolve the symptoms or fails to achieve the treatment goal.…”

Section: Symp Tomatic and Immunosuppre Ss Ive Tre Atmentsmentioning

confidence: 99%

“…Treatment can also be started at low doses (5-10 mg) with an increasing schedule. 13 Chinese guidelines also recommend an increasing regimen, starting at a dose of 20 mg up to 60-80 mg/ day. 14 Japanese guidelines make the following remark: "Long-term oral steroids cause a high incidence of adverse reactions, as well as worsened QOL and mental health; therefore, high doses should be avoided".…”

Section: Symp Tomatic and Immunosuppre Ss Ive Tre Atmentsmentioning

confidence: 99%

“…The dose of azathioprine should be increased to achieve a maintenance dose of 2-3 mg/ kg. 8,10,12,13 Recommended ISs other than azathioprine include cyclosporine, mycophenolate mofetil, methotrexate, and tacrolimus.…”

Section: Symp Tomatic and Immunosuppre Ss Ive Tre Atmentsmentioning

confidence: 99%

“…clinical guidelines, diagnostic criteria, myasthenia gravis guidelines, 12 Italian recommendations, 13 and Chinese guidelines 14 were reviewed. The compilation of new Japanese clinical guidelines for MG is currently underway.…”

Section: Introductionmentioning

confidence: 99%

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Seeking ideal clinical guidelines for myasthenia gravis

Murai

Utsugisawa

Motomura

et al. 2020

Clinical & Exp Neuroim

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Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction (NMJ). Antibodies targeting molecules located in the postsynaptic membrane of NMJs such as the acetylcholine receptor (AChR), 1 muscle-specific receptor tyrosine kinase (MuSK), 2 and low-density lipoprotein receptor-related protein 4 (Lrp4) 3 are thought to be involved in the pathogenesis of the disease. Among these, anti-AChR and anti-MuSK antibodies are considered as pathogens worldwide, whereas the status of anti-Lrp4 is still controversial. It is estimated that there are approximately 29,000 patients with MG in Japan. This figure is twice that found in data from a 2006 national epidemiological study. 4 In Japan, the most recent version of clinical guidelines for MG was published in 2014 (original in Japanese). 5,6 The main feature of the 2014 guidelines is that they prioritize the health-related quality of life (QOL). 7 To ensure a better QOL, the treatment goal was set at minimal manifestation (MM) status or better, with an oral prednisolone (PSL) dose of 5 mg/day or less, and they insisted on the administration of a low dose of oral prednisolone instead of a high dose. They also proposed a strategy called early fast-acting treatment (EFT). After the 2014 guidelines were published, the proportion of MG patients who achieved treatment goals increased. Herein, we searched international and other nations' guidelines/guidance for MG and compared them with the 2014 Japanese guidelines.

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Section: Tre Atment G Oal Smentioning

confidence: 99%

Section: Symp Tomatic and Immunosuppre Ss Ive Tre Atmentsmentioning

confidence: 99%

Section: Symp Tomatic and Immunosuppre Ss Ive Tre Atmentsmentioning

confidence: 99%

Section: Symp Tomatic and Immunosuppre Ss Ive Tre Atmentsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Seeking ideal clinical guidelines for myasthenia gravis

Murai

Utsugisawa

Motomura

et al. 2020

Clinical & Exp Neuroim

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Human Leukocyte Antigen Class II associations in late‐onset Myasthenia Gravis

Spagni

Todi

Monte

et al. 2021

Ann Clin Transl Neurol

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Objective Genetic factors predisposing to late‐onset myasthenia gravis (LOMG) have not been clearly defined yet. However, genome‐wide association studies identified Human Leukocyte Antigen (HLA) Class II alleles as a hotspot in this disease subtype. The aim of this study was to analyze the correlations of HLA Class II alleles with clinical data and titin antibodies in this patient subgroup. Methods This study consecutively enrolled anti‐acetylcholine receptor antibody‐positive, non‐thymoma patients with generalized LOMG. All patients were of Italian ancestry. HLA‐DRB1 and ‐DQB1 genotyping and serum titin antibody testing were performed in this population. Results A total of 107 patients (females: 28/107, 26.2%; median age of onset: 68 years, range: 50‐92) were included. We found a positive association with HLA‐DRB1*07 (P = 1.1 × 10‐5), HLA‐DRB1*14 (P = 0.0251) and HLA‐DQB1*02 (P = 0.0095). HLA‐DRB1*03, HLA‐DRB1*11, and HLA‐DQB1*03 were protective alleles (P = 7.9 × 10‐5, P = 0.0104, and P = 0.0067, respectively). By conditional haplotype analysis, HLA‐DRB1*07‐DQB1*02 was found to be the major risk haplotype (OR = 4.10; 95% C.I.: 2.80‐5.99; P = 6.01 × 10‐11). The mean age at onset was 73.4 years in DRB1*07 homozygotes, 69.7 years in heterozygotes, and 66.6 in non‐carriers (P = 0.0488). DRB1*07 carriers and non‐carriers did not differ in disease severity and response to therapy. Titin antibodies were detected in 61.4% of the cases, having no association with HLA alleles or specific clinical characteristics. Interpretation In our study, we identified the HLA DRB1*07‐DQB1*02 haplotype as a predisposing factor for the development of generalized LOMG in the Italian population.

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