Thymectomy in Juvenile Myasthenia Gravis Is Safe Regarding Long Term Immunological Effects

Popperud, Trine Haug; Gul, Kiran Aftab; Brunborg, Cathrine; Olaussen, Richard W.; Abrahamsen, Tore G.; Osnes, Liv; Kerty, Emila

doi:10.3389/fneur.2021.596859

Cited by 7 publications

(8 citation statements)

References 25 publications

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“…Drawing a parallel between the population of thymectomized individuals studied here and thymectomy performed in patients with other clinical conditions might be enlightening. For instance, patients with myasthenia gravis (MG) treated with thymectomy have been previously studied, and similar results have been observed in the long term: a significant reduction in the number of TRECs, lower numbers of naïve CD4+ and CD8+ T cells along with an increased proportion of memory CD4+ T cells ( 48 ), decreased T cell counts and a reduced TCR repertoire ( 49 ). Although this comparison must be carefully interpreted because patients with MG are mainly adults and have differences in immunological maturity, converging findings from both populations strengthen the evidence for premature immunosenescence in the T cell compartment of thymectomized individuals.…”

Section: Discussionmentioning

confidence: 68%

Early Thymectomy Is Associated With Long-Term Impairment of the Immune System: A Systematic Review

et al. 2021

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Background and AimsCongenital heart diseases (CHDs) are diagnosed in approximately 9 in 1,000 newborns, and early cardiac corrective surgery often requires partial or complete thymectomy. As the long-term effect of early thymectomy on the subsequent development of the immune system in humans has not been completely elucidated, the present study aimed to evaluate the effects of thymus removal on the functional capacity of the immune system after different periods.MethodsA systematic review of the literature was performed using MEDLINE, EMBASE, LILACS and Scopus. The inclusion criteria were original studies that analyzed any component of the immune system in patients with CHD who had undergone thymectomy during cardiac surgery in the first years of life. The results were evaluated for the quality of evidence.ResultsTwenty-three studies were selected and showed that patients who underwent a thymectomy in the first years of life tended to exhibit important alterations in the T cell compartment, such as fewer total T cells, CD4+, CD8+, naïve and CD31+ T cells, lower TRECs, decreased diversity of the TCR repertoire and higher peripheral proliferation (increased Ki-67 expression) than controls. However, the numbers of memory T cells and Treg cells differed across the selected studies.ConclusionsEarly thymectomy, either partial or complete, may be associated with a reduction in many T cell subpopulations and TCR diversity, and these alterations may persist during long-term follow-up. Alternative solutions should be studied, either in the operative technique with partial preservation of the thymus or through the autograft of fragments of the gland.Systematic Review RegistrationProspero [157188].

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Section: Discussionmentioning

confidence: 68%

Early Thymectomy Is Associated With Long-Term Impairment of the Immune System: A Systematic Review

et al. 2021

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“…1,863 of 18,337 MG patients (10.2%) were found to have other autoimmune diseases, the incidence similar to the JMG group (13.6%). Thyroid disease was the most common autoimmune comorbidity in MG patients ( 6 ), also in the JMG group. There was no clear trend toward clustering in other autoimmune diseases ( Figure 3 ).…”

Section: Discussionmentioning

confidence: 99%

“…Thymectomy is an important treatment option for adults with myasthenia gravis when medical therapy is refractory, but remains controversial in the juvenile myasthenia gravis population. Although several recent studies have shown the benefits of thymectomy in JMG patients ( 6 , 36 , 37 ), they are all retrospective and have limitations. And it was reported that some patients presented a crisis after thymectomy, requiring hospitalization and ventilatory support ( 38 ).…”

Section: Discussionmentioning

confidence: 99%

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Outcome and clinical features in juvenile myasthenia gravis: A systematic review and meta-analysis

Lin

Kuang²,

Li³

et al. 2023

Front. Neurol.

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BackgroundJuvenile myasthenia gravis (JMG) is a rare autoimmune disease that has so far only been described in small cohort studies. We defined the clinical characteristics, management, and outcomes of JMG patients over the past 22 years.MethodsA search of PubMed, EMBASE, and web of science (1/2000–2/2022) identified all English language and human studies of JMG. The population was patients diagnosed with JMG. Outcomes included the history of myasthenic crisis, autoimmune comorbidity, mortality, and treatment outcome. Data extraction was performed by independent reviewers. And we performed a pooled reanalysis of all published data in the included studies and compared with other studies of adult cohorts.ResultsWe identified 11 articles describing 1,109 patients diagnosed between 2006 and 2021. JMG occurred in 60.4% of female patients. The mean age at presentation was 7.38 years old, and 60.6% of the patients had ocular symptoms as the first clinical manifestation. The most common initial presentation was ptosis, which occurred in 77.7% patients. AchR-Ab positive accounted for 78.7%. 641 patients received thymus examination, found to have thymic hyperplasia in 64.9% and thymoma in 2.2%. Autoimmune comorbidity was found in 13.6% and the most common one is thyroid disease (61.5%). First-line therapy, including pyridostigmine and steroids, was initiated in 97.8 and 68.6%, respectively. Six patients resolved spontaneously without treatment. Thymectomy was performed in 45.6%. 10.6% of patients had a history of myasthenic crisis. Completely stable remission was achieved in 23.7% and mortality was reported in 2 studies, which reported 8 deaths.ConclusionJMG is a rare disease with a relatively benign course, and differs from adult MG in some clinical features. The treatment regimen guideline for children is still not well-established. There is a need for prospective studies to properly evaluate treatment regimes.

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“…The concern with a thymectomy in a child or juvenile patient with a still developing immune system is that the absence of a thymus could have negative long-term consequences for the immune responses later-on in life. Popperud et al followed up on this question by analyzing T-cell subsets in thymectomized JMG patients (median age of 17 at thymectomy) [33]. Seven to 26 years after thymectomy they found a lower number of naive CD4 þ helper T-cells and CD8 cytotoxic T-cell subsets and an increase in memory T-cells indicating a premature ageing of the immune system, However, negative clinical consequences could not be discerned.…”

Section: Juvenile Onset Myasthenia Gravis and Thymectomymentioning

confidence: 99%

Thymectomy in myasthenia gravis

Rath,

Moser,

Zimprich

2023

Current Opinion in Neurology

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Purpose of review Thymectomy has long been used in the treatment of patients with myasthenia gravis and antibodies against the acetylcholine receptor. However, its effectiveness has only been proven a few years ago in a randomized controlled trial in patients under the age of 65. Here, we review the current literature focusing on patient subgroups, potential biomarkers for outcome prediction and the choice of surgical approach. Recent findings Long-term follow-up studies after thymectomy confirmed that the benefits regarding clinical outcome parameters and a reduced need for immunosuppressive treatment persist. Nevertheless, a substantial proportion of patients in real-world cohorts do not reach complete stable remission after thymectomy indicating that the underlying autoimmune process is sustained in the periphery. Our understanding of the responsible mechanisms has improved with recent studies. Presently, outcome data after thymectomy in several patient subgroups, such as those aged over 50 years, those with juvenile onset or those with purely ocular symptoms are limited and have been the focus of recent research activities. Similarly, biomarkers guiding an appropriate patient selection for thymectomy are under investigation. A number of cohort studies demonstrated that minimal invasive surgical techniques such as extended robotic thymectomy lead to similar positive outcomes as a transsternal approach with potentially fewer short-term adverse effects. Summary Thymectomy is an effective treatment option in adult patients with early onset acetylcholine-receptor positive myasthenia gravis but uncertainty remains with regard to certain patient subgroups.

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Thymectomy in Juvenile Myasthenia Gravis Is Safe Regarding Long Term Immunological Effects

Cited by 7 publications

References 25 publications

Early Thymectomy Is Associated With Long-Term Impairment of the Immune System: A Systematic Review

Early Thymectomy Is Associated With Long-Term Impairment of the Immune System: A Systematic Review

Outcome and clinical features in juvenile myasthenia gravis: A systematic review and meta-analysis

Thymectomy in myasthenia gravis

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