ArticleMuscle & nerve2020
Clinical features of LRP4/agrin-antibody-positive myasthenia gravis: A multicenter study.
Article in Muscle & nerve, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers, 2 of them syntheses that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
40 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Clinical Characteristics and Treatment Management of Seronegative Myasthenia Gravis: A Systematic Review of the Literature.Muscle & nerve · 2026Pooled it
- Role of Agrin in tissue repair and regeneration: From mechanisms to therapeutic opportunities (Review).International journal of molecular medicine · 2024Pooled it
- Review
- Comprehensive review on myasthenia gravis: patho-physiology, diagnosis, management strategies.Wiener medizinische Wochenschrift (1946) · 2026Review
- Article
- Low Prevalence and Inconsistency of LRP4-IgG Detection in Suspected Myasthenia Gravis: A Multicenter CBA Comparison.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Diagnostic Challenges of LRP4 Antibody Testing in Myasthenia Gravis: A Case Series of Four LRP4 Positive Cases With Uncertain Diagnostic Significance.Case reports in neurological medicine · 2026Article
- Immunological Targets in Generalized Myasthenia Gravis Treatment: Where Are We Going Now?Brain sciences · 2025Review
- Effective use of BCMA-targeting bispecific T cell-engaging antibody in treatment-refractory LRP4Molecular therapy : the journal of the American Society of Gene Therapy · 2025Article
- Review
- Toward European harmonization of national myasthenia gravis registries: modified Delphi procedure-based expert consensus on collectable data.Orphanet journal of rare diseases · 2025Article
- Development and Application of a Cell-Based Assay for Detecting Anti-Agrin Antibodies Associated With Myasthenia Gravis.Journal of clinical neurology (Seoul, Korea) · 2025Article
- Unusual Presentation of Double-seronegative Myasthenia Gravis with Positive Anti-LRP4 Antibody: Diagnostic Utility of a Videofluoroscopic Swallowing Study.Internal medicine (Tokyo, Japan) · 2025Article
- Clinical characteristics of myasthenia gravis patients with coexistence of AChR and titin antibodies.Frontiers in neurology · 2025Article
- Complement and MHC patterns can provide the diagnostic framework for inflammatory neuromuscular diseases.Acta neuropathologica · 2024Review
- Triple-seronegative myasthenia gravis: clinical and epidemiological characteristics.Arquivos de neuro-psiquiatria · 2024Article
- A Focus on Myasthenic Ptosis: The Interface of Medical and Surgical Treatment.World journal of plastic surgery · 2024Review
- Advancements and prospects of novel biologicals for myasthenia gravis: toward personalized treatment based on autoantibody specificities.Frontiers in pharmacology · 2024Review
- Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background.Pathophysiology : the official journal of the International Society for Pathophysiology · 2023Review
- Electrophysiological evaluation of the neuromuscular junction: a brief review.Arquivos de neuro-psiquiatria · 2023Review
Corrections and comments
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Authors and funding
27 authors.
Funding
Abstract
introductionOur aim in this study was to identify the prevalence and clinical characteristics of LRP4/agrin-antibody-positive double-seronegative myasthenia gravis (DNMG).
methodsDNMG patients at 16 sites in the United States were tested for LRP4 and agrin antibodies, and the clinical data were collected.
resultsOf 181 DNMG patients, 27 (14.9%) were positive for either low-density lipoprotein receptor-related protein 4 (LRP4) or agrin antibodies. Twenty-three DNMG patients (12.7%) were positive for both antibodies. More antibody-positive patients presented with generalized symptoms (69%) compared with antibody-negative patients (43%) (P ≤ .02). Antibody-positive patients' maximum classification on the Myasthenia Gravis Foundation of America (MGFA) scale was significantly higher than that for antibody-negative patients (P ≤ .005). Seventy percent of antibody-positive patients were classified as MGFA class III, IV, or V compared with 39% of antibody-negative patients. Most LRP4- and agrin-antibody-positive patients (24 of 27, 89%) developed generalized myathenia gravis (MG), but with standard MG treatment 81.5% (22 of 27) improved to MGFA class I or II during a mean follow-up of 11 years. DISCUSSION: Antibody-positive patients had more severe clinical disease than antibody-negative patients. Most DNMG patients responded to standard therapy regardless of antibody status.
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