{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T14:28:12Z","timestamp":1772288892081,"version":"3.50.1"},"reference-count":26,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T00:00:00Z","timestamp":1753920000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Highly active myasthenia gravis refers to a subset of refractory patients who exhibit recurrent exacerbations and crises. Eculizumab, a complement C5 inhibitor, has shown its efficacy and safety for patients with anti-acetylcholine receptor antibody-positive(AchR +)refractory generalized myasthenia gravis(gMG) in the REGAIN trial. However, the efficacy and safety of eculizumab in treating MG patients with severe infections have not yet been supported by clinical evidence. This is a case series reporting four patients with highly active myasthenia gravis complicated by severe infections. Changes in Myasthenia Gravis-Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) scores were recorded before and after 12 injections of eculizumab to assess efficacy. Pathogen characteristics of infections were summarized using bacterial culture and next-generation sequencing (NGS) results, presented as a heatmap to illustrate pathogen species and abundance. Inflammatory markers, including Procalcitonin (PCT), C-Reactive Protein (CRP), neutrophil count, and total lymphocyte count, were monitored to evaluate the safety. Treatment regimens were retrospectively analyzed to further assess clinical outcomes and safety. The baseline ADL data for the four patients was 22 \u00b1 2.31 (Mean \u00b1 SD), and the baseline QMG data was 30.5 \u00b1 8.23. After 12 injections of eculizumab treatment, the scores decreased to ADL 4.75 \u00b1 3.3 and QMG 14 \u00b1 3.37. During the treatment, no apparent worsening of infections related to Eculizumab was noted. Three patients successfully had their tracheostomy tubes removed, and none of the four patients experienced further myasthenic crises. Eculizumab demonstrated clinical improvement in this series, and the treatment was well-tolerated. This case series addresses the need for data on complement inhibitors in highly active myasthenia gravis patients with severe infections, provides clinical reference support for the expanded application of eculizumab.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1596283","type":"journal-article","created":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T05:39:13Z","timestamp":1753940353000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: Eculizumab in highly active myasthenia gravis complicated by severe infections"],"prefix":"10.3389","volume":"16","author":[{"given":"Yufei","family":"Deng","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haocheng","family":"Luo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chaoyue","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shuangshuang","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xuxiang","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xianni","family":"Yan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaojun","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qilong","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,7,31]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1038\/s41572-019-0079-y","article-title":"Myasthenia gravis","volume":"5","author":"Gilhus","year":"2019","journal-title":"Nat Rev Dis Primers"},{"key":"B2","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1002\/mus.27832","article-title":"Myasthenic crisis","volume":"68","author":"Claytor","year":"2023","journal-title":"Muscle Nerve"},{"key":"B3","doi-asserted-by":"publisher","first-page":"17562864231213240","DOI":"10.1177\/17562864231213240","article-title":"Guideline for the management of myasthenic syndromes","volume":"16","author":"Wiendl","year":"2023","journal-title":"Ther Adv Neurol Disord"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1007\/s00281-021-00895-4","article-title":"The emerging role of complement in neuromuscular disorders","volume":"43","author":"Lee","year":"2021","journal-title":"Semin Immunopathol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1111\/nyas.13522","article-title":"Myasthenia gravis: the role of complement at the neuromuscular junction","volume":"1412","author":"Howard","year":"2018","journal-title":"Ann New York Acad Sci"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(17)30369-1","article-title":"Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study","volume":"16","author":"Howard","year":"2017","journal-title":"Lancet Neurol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.3389\/fcimb.2022.1016728","article-title":"Pneumonia in myasthenia gravis: Microbial etiology and clinical management","volume":"12","author":"Su","year":"2022","journal-title":"Front Cell Infect Microbiol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-018-8751-9","article-title":"Myasthenia gravis and infectious disease","volume":"265","author":"Gilhus","year":"2018","journal-title":"J Neurol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002790","article-title":"International consensus guidance for management of myasthenia gravis: Executive summary","volume":"87","author":"Sanders","year":"2016","journal-title":"Neurology"},{"key":"B10","unstructured":"Drugs@FDA: FDA-approved drugs"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.15585\/mmwr.mm6627e1","article-title":"High risk for invasive meningococcal disease among patients receiving eculizumab (Soliris) despite receipt of meningococcal vaccine","volume":"66","author":"McNamara","year":"2017","journal-title":"MMWR Morb Mortal Wkly Rep"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2020.00538","article-title":"Novel treatments in myasthenia gravis","volume":"11","author":"Menon","year":"2020","journal-title":"Front Neurol"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410410615","article-title":"Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis","volume":"41","author":"Gajdos","year":"1997","journal-title":"Myasthenia gravis Clin study Group Ann Neurol"},{"key":"B14","doi-asserted-by":"publisher","first-page":"17562864241309431","DOI":"10.1177\/17562864241309431","article-title":"Eculizumab in thymoma-associated myasthenia gravis: A real-world cohort study","volume":"17","author":"Jin","year":"2024","journal-title":"Ther Adv Neurol Disord"},{"key":"B15","doi-asserted-by":"publisher","first-page":"17562864241261602","DOI":"10.1177\/17562864241261602","article-title":"The safety and efficacy profile of eculizumab in myasthenic crisis: a prospective small case series","volume":"17","author":"Song","year":"2024","journal-title":"Ther Adv Neurol Disord"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-023-06900-y","article-title":"Starting eculizumab as rescue therapy in refractory myasthenic crisis","volume":"44","author":"Vinciguerra","year":"2023","journal-title":"Neurol Sci"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-021-10428-6","article-title":"Complement inhibition initiated recovery of a severe myasthenic crisis with COVID-19","volume":"268","author":"Hofstadt-van Oy","year":"2021","journal-title":"J Neurol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.696385","article-title":"Eculizumab pharmacokinetics and pharmacodynamics in patients with generalized myasthenia gravis","volume":"12","author":"Monteleone","year":"2021","journal-title":"Front Neurol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"798","DOI":"10.1002\/mus.27973","article-title":"Activation of the classical complement pathway in myasthenia gravis with acetylcholine receptor antibodies","volume":"68","author":"Ozawa","year":"2023","journal-title":"Muscle Nerve"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1097\/01.CCM.0000181731.53912.D9","article-title":"Clinical and economic consequences of ventilator-associated pneumonia: a systematic review","volume":"33","author":"Safdar","year":"2005","journal-title":"Crit Care Med"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/ciy543","article-title":"A systematic review and meta-analysis of ventilator-associated pneumonia in adults in asia: an analysis of national income level on incidence and etiology","volume":"68","author":"Bonell","year":"2019","journal-title":"Clin Infect Dis"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1128\/aac.00053-22","article-title":"Candida auris pan-drug-resistant to four classes of antifungal agents","volume":"66","author":"Jacobs","year":"2022","journal-title":"Antimicrob Agents Chemother"},{"key":"B23","doi-asserted-by":"publisher","first-page":"878","DOI":"10.3390\/jof7100878","article-title":"International multicentre study of candida auris infections","volume":"7","author":"Pandya","year":"2021","journal-title":"J Fungi (Basel)"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1006290","article-title":"Candida auris: A rapidly emerging cause of hospital-acquired multidrug-resistant fungal infections globally","volume":"13","author":"Chowdhary","year":"2017","journal-title":"PLoS Pathog"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1159\/000491439","article-title":"Complement and bacterial infections: From molecular mechanisms to therapeutic applications","volume":"10","author":"Heesterbeek","year":"2018","journal-title":"J Innate Immun"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1016\/j.molimm.2011.05.001","article-title":"Complement deficiency states and associated infections","volume":"48","author":"Skattum","year":"2011","journal-title":"Mol Immunol"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1596283\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T05:39:14Z","timestamp":1753940354000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1596283\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,31]]},"references-count":26,"alternative-id":["10.3389\/fimmu.2025.1596283"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2025.1596283","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7,31]]},"article-number":"1596283"}}