Clinical characteristics of <scp>anti‐AChR‐MuSK‐LRP4</scp> antibody‐negative myasthenia gravis in China

Li, Wenbo; Li, Peipei; Cui, Weike; Wang, Shumin; Ji, Ying; Zhang, Linyuan; He, Xiaoxiao; Zhou, Shuxian; Shen, Tong; Zhao, Xue; Lv, Jie; Zhang, Yingna; Zhang, Jing; Fang, Hua; Yang, Jing; Zhang, Yunke; Cui, Xin; Zhang, Qingyong; Gao, Feng

doi:10.1002/mus.27822

Cited by 2 publications

(3 citation statements)

References 32 publications

(76 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Whether efgartigimod can improve the symptoms of triple-seronegative MG needs further study. Triple-seronegative MG has shown positive response to steroids and pyridostigmine, with approximately a third of patients achieving either complete stable remission or pharmacological remission (9). After 3-month treatment with pyridostigmine in combination with immunosuppressive therapy, the present patient achieved minimal symptom expression, which was defined as MG-ADL score of 0 or 1 and regarded as an practical tool for MG treatment goals (12).…”

Section: Discussionmentioning

confidence: 82%

“…The diagnosis and treatment are more challenging in tripleseronegative MG. As no detectable specific antibodies, diagnosis of triple-seronegative MG should be supported by adequate clinical characteristics and electrophysiological support, especially when the effectiveness of standard treatment is dissatisfactory. From a clinical point of view, it is observed that the clinical characteristics of triple-seronegative MG patients varied among different age groups (9). The overall prognosis of triple-seronegative MG is relatively good, with a low occurrence of crisis.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Case report: Coexistence of triple-seronegative myasthenia gravis and pathology-proven cryptogenic organizing pneumonia

Huang,

Wang,

Gao

et al. 2023

Front. Neurol.

View full text Add to dashboard Cite

ObjectiveEmerging evidence shows that patients with myasthenia gravis (MG) were at a higher risk for the co-occurrence of other autoimmune diseases, which reflects phenotypic heterogeneity in MG. The coexistence of MG and cryptogenic organizing pneumonia (COP) has rarely been reported. The present case is to report the coexistence of triple-seronegative MG and pathology-proven COP in a patient.MethodsThe clinical data of the patient were derived from medical records of Nanjing First Hospital, Nanjing Medical University, China. Written informed consent was obtained from the patient.ResultsWe presented a 56-year-old man with acute respiratory syndrome, who was diagnosed with COP based on the intra-alveolar fibroinflammatory buds (Masson's bodies) in the pathology of bronchoscopy biopsy. Oral prednisone induced dramatic symptomatic improvement and complete resolution of previous lung lesions. After a stable course of no respiratory symptom for 2 months, he was referred to the neurology department with complaints of fluctuating generalized muscle weakness. He was diagnosed with triple-seronegative MG based on fluctuating weakness, neostigmine test-positivity and RNS-positivity. After three-month treatment with pyridostigmine in combination with tacrolimus, the symptoms gradually improved and he achieved minimal symptom expression.ConclusionsThis case highlights the rare coexistence of triple-seronegative MG and pathology-proven COP. However, a causal association between COP and MG cannot be explicitly ascertained. In future, more data are needed to clarify the relationship, taking into account the limited number of cases reported with this coexistence of the diseases.

show abstract

Section: Discussionmentioning

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Case report: Coexistence of triple-seronegative myasthenia gravis and pathology-proven cryptogenic organizing pneumonia

Huang,

Wang,

Gao

et al. 2023

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…Significant findings included a bimodal distribution of the age of onset (0–9 y/o and 40–49), coexisting thymic hyperplasia, and a generally favorable prognosis. Adult patients were more likely to have generalization from ocular forms, while juvenile subjects were more likely to experience clinical relapse [ 21 ].…”

Section: Epidemiological and Clinical Aspectsmentioning

confidence: 99%

Diagnosis and Management of Seronegative Myasthenia Gravis: Lights and Shadows

Vinciguerra,

Bevilacqua,

Lupica

et al. 2023

Brain Sciences

View full text Add to dashboard Cite

Myasthenia gravis (MG) is an antibody-mediated neuromuscular disease affecting the neuromuscular junction. In most cases, autoantibodies can be detected in the sera of MG patients, thus aiding in diagnosis and allowing for early screening. However, there is a small proportion of patients who have no detectable auto-antibodies, a condition termed “seronegative MG” (SnMG). Several factors contribute to this, including laboratory test inaccuracies, decreased antibody production, immunosuppressive therapy, immunodeficiencies, antigen depletion, and immune-senescence. The diagnosis of SnMG is more challenging and is based on clinical features and neurophysiological tests. The early identification of these patients is needed in order to ensure early treatment and prevent complications. This narrative review aims to examine the latest updates on SnMG, defining the clinical characteristics of affected patients, diagnostic methods, management, and therapeutic scenarios.

show abstract

Clinical characteristics of anti‐AChR‐MuSK‐LRP4 antibody‐negative myasthenia gravis in China

Cited by 2 publications

References 32 publications

Case report: Coexistence of triple-seronegative myasthenia gravis and pathology-proven cryptogenic organizing pneumonia

Case report: Coexistence of triple-seronegative myasthenia gravis and pathology-proven cryptogenic organizing pneumonia

Diagnosis and Management of Seronegative Myasthenia Gravis: Lights and Shadows

Contact Info

Product

Resources

About