Role of the thymus in autoimmune myasthenia gravis

Berrih‐Aknin, Sonia

doi:10.1111/cen3.12319

Cited by 22 publications

(11 citation statements)

References 97 publications

(118 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The role of the thymus in MG pathogenesis is supported by a number of factors, including the high rates of thymic pathology seen in MG patients, correlation between anti-AChR antibody levels and the degree of follicular hyperplasia and favorable outcomes post-thymectomy ( 73 ). Thymic hyperplasia is not uncommon in JMG but thymoma is rare as demonstrated by a number of large cohort studies ( 22 , 74 , 75 ).…”

Section: Treatment Of Jmgmentioning

confidence: 99%

Management of Juvenile Myasthenia Gravis

2020

View full text Add to dashboard Cite

Juvenile Myasthenia Gravis (JMG) is a rare disorder, defined as myasthenia gravis in children younger than 18 years of age. While clinical phenotypes are similar to adults, there are a number of caveats that influence management: broader differential diagnoses; higher rates of spontaneous remission; and the need to initiate appropriate treatment early, to avoid the long-term physical and psychosocial morbidity. Current practice is taken from treatment guidelines for adult MG or individual experience, with considerable variability seen across centers. We discuss our approach to treating JMG, in a large specialist JMG service, and review currently available evidence and highlight potential areas for future research. First-line treatment of generalized JMG is symptomatic management with pyridostigmine, but early use of immunosuppression, where good control is not achieved is important. Oral prednisolone is used as first-line immunosuppression with appropriate prevention and monitoring of side effects. Second-line therapies including azathioprine and mycophenolate may be considered where there is: no response to steroids, inability to wean to a reasonable minimum effective dose or if side-effects are intolerable. Management of ocular JMG is similar, but requires close involvement of ophthalmology in young children to prevent amblyopia. Muscle-specific tyrosine kinase (MuSK)-JMG show a poorer response to pyridostigmine and anecdotal evidence suggests that rituximab should be considered as second-line immunosuppression. Thymectomy is indicated in any patient with a thymoma, and consideration should be given in acetylcholine receptor (AChR) positive JMG allowing time for spontaneous remission. The benefit is less clear in ocular JMG and is not advised in MuSK-JMG. Children experiencing a myasthenic crisis require urgent hospital admission with access to the intensive care unit. PLEX is preferred over IVIG due to rapid onset of action, but this needs to be balanced with feasibility in very young children. Key questions remain in the management of JMG: when to initiate both first- and second-line treatments, choosing between steroid-sparing agents, and determining the optimal dose and treatment duration. We feel that given the rarity of this disease, the establishment of national registries and collaboration across groups will be needed to address these issues and facilitate future drug trials in JMG.

show abstract

Section: Treatment Of Jmgmentioning

confidence: 99%

Management of Juvenile Myasthenia Gravis

2020

View full text Add to dashboard Cite

show abstract

“…Similarly to thyroiditis, in MG, several antigens are the targets of the autoantibodies, and the disease features depend upon the nature of the antibodies. Patients with anti-AChR, but not with anti-MuSK antibodies, have thymic pathologies, hyperplasia among the young patients, and thymoma among the oldest patients ( 60 ).…”

Section: Differential and Common Features In Atds And Mgmentioning

confidence: 99%

Autoimmune Thyroiditis and Myasthenia Gravis

Lopomo

Berrih‐Aknin

2017

Front. Endocrinol.

Self Cite

View full text Add to dashboard Cite

Autoimmune diseases (AIDs) are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as “self” are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD) is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto’s thyroiditis and Graves disease. They are frequently associated with other organ or non-organ specific AIDs, such as myasthenia gravis (MG). In fact, ATD seems to be the most associated pathology to MG. The etiology of both diseases is multifactorial and it is due to genetic and environmental factors, and each of them has specific characteristics. The two pathologies show many commonalities, such as the organ-specificity with a clear pathogenic effect of antibodies, the pathological mechanisms, such as deregulation of the immune system and the implication of the genetic predisposition. They also show some differences, such as the mode of action of the antibodies and therapies. In this review that focuses on ATD and MG, the common features and the differences between the two diseases are discussed.

show abstract

“…TFH is commonly seen adjacent to thymoma and is likely a major contributor to the elevated AChR antibodies 26 . An intriguing question that arises is whether the MG outcome depends on the presence of TFH associated with thymoma.…”

Section: Discussionmentioning

confidence: 99%

Thymoma pathology and myasthenia gravis outcomes

et al. 2021

View full text Add to dashboard Cite

Introduction: There is limited evidence regarding the impact of World Health Organization (WHO) subtype of thymoma on post-thymectomy outcome of thymoma-associated myasthenia gravis (TAMG). The objective was to determine if the pathological subtypes of thymoma were associated with post-thymectomy outcomes of myasthenia gravis (MG), in patients with TAMG.Methods: We performed a retrospective study of consecutive patients with TAMG who attended the neuromuscular clinic between January 2018 and December 2019 with a minimum follow-up of 1 y after thymectomy. Outcome measures were MG Impairment Index (MGII), single-simple question (SSQ), Myasthenia Gravis Foundation of America post-intervention status (MGFA PIS) and non-responder MG status at last assessment.Results: Ninety-five patients were included; mean age at onset was 48.1 ± 12.1 y; 54(56.8%) were females. Thirteen patients developed MG post-thymectomy. The most common thymoma was WHO type B2 in 39 (41.1%). Most patients (40, 42.1%) had Masaoka stage II thymoma. There was no association of thymoma subtypes or Masaoka stage of disease with age, gender, MG phenotype, serology, postthymectomy onset, interval from onset to thymectomy, MGII, SSQ, MGFA PIS, or non-responder status. Associations were found between positive serology and lower

show abstract

Role of the thymus in autoimmune myasthenia gravis

Cited by 22 publications

References 97 publications

Management of Juvenile Myasthenia Gravis

Management of Juvenile Myasthenia Gravis

Autoimmune Thyroiditis and Myasthenia Gravis

Thymoma pathology and myasthenia gravis outcomes

Contact Info

Product

Resources

About