Auto-anticorps LRP4 dans la myasthénie grave double séronégative: une revue systématique.

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Auto-anticorps LRP4 dans la myasthénie grave double séronégative: une revue systématique.

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:arrow: Lu sur :https://www.ncbi.nlm.nih.gov/pubmed/29334041

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Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Double Seronegative Myasthenia Gravis: A Systematic Review.
Bacchi S1, Kramer P2, Chalk C3.
Can J Neurol Sci. 2018 Jan;45(1):62-67. doi: 10.1017/cjn.2017.253.


Abstract
  • BACKGROUND:

    Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which a clinical diagnosis may be confirmed with serological testing. The most common autoantibodies used to support a diagnosis of MG are anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies. In cases in which both of these autoantibodies are negative (termed double-seronegative [dSNMG]), other autoantibodies such as low-density lipoprotein receptor-related protein 4 (LRP4) may be used to aid in diagnosis.
  • METHODS:

    We have undertaken a systematic literature review to identify studies that have assessed the frequency of anti-LRP4 antibodies in dSNMG patients and the characteristics of anti-LRP4+ dSNMG patients (epidemiology, clinical features, electromyographic findings, or management). PubMed, EMBASE, Medline, and Scopus were searched on January 14, 2017, using the medical subject headings "myasthenia gravis" and "low-density lipoprotein receptor-related protein 4" or "LRP4."
  • RESULTS:

    The initial search identified 367 articles. Fourteen publications met the inclusion criteria. There were ten cross-sectional research studies, three were case series, and one was a case report. The majority of studies were limited by small sample sizes of LRP4+ dSNMG. There has been a wide range of frequencies of anti-LRP4 antibodies detected in different MG patient populations, some involving different laboratory techniques.
  • CONCLUSIONS:

    LRP4+ dSNMG is more likely than LRP4- dSNMG to have a younger onset of disease and occur in females. LRP4+ dSNMG most often is mild in severity and often involves isolated ocular weakness. It typically responds well to pyridostigmine or prednisone.

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