Clinical and immunological predictors of prognosis for Japanese patients with thymoma-associated myasthenia gravis

Department of Neurology, Keio University School of Medicine, Tokyo, Japan. Electronic address: .
Journal of neuroimmunology (Impact Factor: 2.47). 04/2013; 258(1-2). DOI: 10.1016/j.jneuroim.2013.03.001
Source: PubMed


There are no immunological markers to predict the prognosis of thymoma-associated myasthenia gravis (MG). Clinical and immunological factors associated with thymoma recurrence or MG relapse were examined by logistic analyses in 56 Japanese patients with thymoma-associated MG. Patients with anti-Kv1.4 antibodies showed higher frequencies of thymoma recurrence and MG relapse compared to those without. Anti-Kv1.4 antibody, Masaoka stage 4, World Health Organization type B3, and adjuvant radiotherapy were associated with thymoma recurrence. Multivariate analyses showed that anti-Kv1.4 antibody was the only independent factor associated with MG relapse. Anti-Kv1.4 antibody is a useful predictor of the prognosis of thymoma-associated MG.

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    ABSTRACT: Pediatric myasthenia gravis (P-MG) is an autoimmune disease affecting the neuromuscular junction (NMJ), and is rare in children. The roadmap of the research carried out for P-MG in Hong Kong over the past 30 years was reviewed. Currently, there is a lack of epidemiological studies in comparing clinical data or the outcome for people of different ethnic origins. P-MG is more common in Asians, especially Chinese and Japanese. Because of the lack of comparison studies for P-MG worldwide, the author initiated collaboration with international colleagues in 2008, and set up “The International Paediatric Myasthenia Gravis Registry Focus Group (FG)” in 2009. We then established an “International Pediatric Myasthenia Gravis Registry (IPMGR)” to review the current knowledge of P-MG in children. We designed an e-MG questionnaire for “Myasthenia Syndromes in Children,” and launched through the website (ICNAPaedia) of the International Child Neurology Association (ICNA) in 2009–2010. As of 2014, we had collected 121 replies from clinicians–researchers in 43 countries. In 2013, we started a Delphi process for obtaining a consensus for diagnosis, management and treatment of P-MG. Despite the lack of research funding support as a result of the rarity of P-MG, and the lack of innovative pharmaceutical research and development, we still stress that we set up a foundation as a small step towards consolidating international collaboration to carry out epidemiological studies, genetic studies and randomized controlled trials of all the current available treatments, especially steroids for ocular P-MG, and indications for thymectomy.
    No preview · Article · Oct 2014 · Clinical and Experimental Neuroimmunology