{
    "abstract": "The long lead time required to diagnose refractory myasthenia gravis (MG) can prevent early attempts at new treatments. Herein, we aimed to predict refractory MG by comparing treatment responses during the early stages between patients with refractory and non-refractory MG.\nWe retrospectively investigated the medical records of patients with acetylcholine receptor antibody-positive generalized MG. Refractory MG was defined as (1) inability to lower prednisolone below 20\u00a0mg/day, (2) requiring regular intravenous immunoglobulin or plasmapheresis, or (3) Myasthenia Gravis Activities of Daily Living score\u2009\u2265\u20096 after treatment with corticosteroids and \u2265\u20091 oral immunosuppressant for \u2265\u200912 months.\nOf 133 patients, 29 (21.8%) and 104 (78.2%) had refractory and non-refractory MG, respectively. The median duration from the diagnosis of MG to that of refractory MG was 31 months. During the first year following diagnosis, the proportion of patients who achieved a lowest effective prednisolone dose of \u2264\u200920\u00a0mg was lower in the refractory group than in the non-refractory group (69.0% vs. 92.0%). Conversely, the proportions of patients who initiated oral immunosuppressive therapy (58.6% vs. 22.5%), required rescue interventions (48.3% vs. 22.1%), or experienced a myasthenic crisis (34.5% vs. 14.4%) were significantly higher in the refractory group. The risk of refractory MG was 18.7-fold higher in patients who failed to achieve lowest effective prednisolone dose under 20\u00a0mg/day and started oral immunosuppressant during the first year than those with neither of these conditions.\nThe refractory group displayed distinct clinical features during the early disease stages, including frequent crises and higher levels of immunosuppressive treatment. These features can be used for early prediction of refractory MG.",
    "authors": [
        {
            "affiliation": "Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.",
            "firstname": "Hee Jo",
            "initials": "HJ",
            "lastname": "Han"
        },
        {
            "affiliation": "Yonsei University College of Medicine, Seoul, Republic of Korea.",
            "firstname": "Susung",
            "initials": "S",
            "lastname": "Kim"
        },
        {
            "affiliation": "Department of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.",
            "firstname": "Hyung Jun",
            "initials": "HJ",
            "lastname": "Park"
        },
        {
            "affiliation": "Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.",
            "firstname": "Ha Young",
            "initials": "HY",
            "lastname": "Shin"
        },
        {
            "affiliation": "Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea. kswneuro@yuhs.ac.",
            "firstname": "Seung Woo",
            "initials": "SW",
            "lastname": "Kim"
        }
    ],
    "conclusions": null,
    "copyrights": "\u00a9 2025. Fondazione Societ\u00e0 Italiana di Neurologia.",
    "doi": "10.1007/s10072-025-08196-6\n10.1002/mus.20950\n10.1177/1756285617749134\n10.1177/1756286419832242\n10.3205/000239\n10.1007/s00415-019-09667-5\n10.1080/14712598.2023.2184257\n10.1038/s41582-018-0110-z\n10.1177/17562864231213240\n10.1001/jamaneurol.2022.2887\n10.1016/s1474-4422(17)30369-1\n10.1111/ene.14675\n10.1002/mus.22140\n10.1212/wnl.0000000000011124\n10.1136/practneurol-2015-001126\n10.1002/acn3.51492\n10.3389/fneur.2021.725700\n10.5582/irdr.2019.01121\n10.1212/wnl.0000000000013121",
    "journal": "Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology",
    "keywords": [
        "Autoimmune disease",
        "Generalized myasthenia gravis",
        "Immunosuppressive agents",
        "Myasthenia gravis",
        "Neuromuscular junction disease"
    ],
    "methods": null,
    "publication_date": "2025-04-26",
    "pubmed_id": "40281192\n18059039\n29403543\n30854027\n23766745\n27790079\n31828474\n36852951\n38152089\n36121672\n29066163\n33306873\n22006686\n33144515\n25977271\n35080153\n34925206\n31890449\n34857535",
    "results": "Of 133 patients, 29 (21.8%) and 104 (78.2%) had refractory and non-refractory MG, respectively. The median duration from the diagnosis of MG to that of refractory MG was 31 months. During the first year following diagnosis, the proportion of patients who achieved a lowest effective prednisolone dose of \u2264\u200920\u00a0mg was lower in the refractory group than in the non-refractory group (69.0% vs. 92.0%). Conversely, the proportions of patients who initiated oral immunosuppressive therapy (58.6% vs. 22.5%), required rescue interventions (48.3% vs. 22.1%), or experienced a myasthenic crisis (34.5% vs. 14.4%) were significantly higher in the refractory group. The risk of refractory MG was 18.7-fold higher in patients who failed to achieve lowest effective prednisolone dose under 20\u00a0mg/day and started oral immunosuppressant during the first year than those with neither of these conditions.",
    "title": "Early prediction of refractory myasthenia gravis based on response to treatment within the first year of diagnosis.",
    "xml": "<Element 'PubmedArticle' at 0x7885033d6c00>"
}