{"doi":"10.1002/mus.27326","title":"The use of eculizumab in ventilator‐dependent myasthenia gravis patients","abstract":"<jats:title>Abstract</jats:title><jats:sec><jats:title>Introduction/Aims</jats:title><jats:p>Eculizumab has been shown to be efficacious in acetylcholine receptor antibody–positive (AChR<jats:sup>+</jats:sup>) Myasthenia Gravis Foundation of America (MGFA) class II, III, and IV generalized myasthenia gravis (gMG) patients. However, it has not been studied in MGFA class V gMG patients.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We report three AChR<jats:sup>+</jats:sup>, refractory, MGFA class V gMG patients treated with eculizumab. MGFA class, MG‐Composite (MGC) score and MG Activities of Daily Living (MG‐ADL) score were assessed before and after eculizumab.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Two of three gMG patients, refractory to intravenous immunoglobulin, plasmapheresis, prednisone, and (in one case) rituximab, showed a robust response to eculizumab with marked improvement in MGFA, MG‐ADL, and MGC measures. The third patient showed a partial response to eculizumab but remained on noninvasive ventilation and gastrostomy intubation. Patients 1 and 2 achieved minimal manifestation status at week 4 and week 6, respectively, and showed continued improvement on MG‐ADL and MGC scores through weeks 55 and 43, respectively, with eculizumab. The third patient showed a partial response at week 10, followed by a slight decline in his MG‐ADL score, but noted a slow but an incomplete improvement afterward on MG‐ADL and MGC scores, possibly due to delayed eculizumab infusion.</jats:p></jats:sec><jats:sec><jats:title>Discussion</jats:title><jats:p>Eculizumab may play a role in the treatment of patients with MGFA class V, refractory gMG. Larger studies are required to provide further evidence.</jats:p></jats:sec>","journal":"Muscle &amp; Nerve","year":2021,"id":645764,"datarank":0.5570358100056463,"base_score":3.713572066704308,"endowment":3.713572066704308,"self_citation_contribution":0.5570358100056463,"citation_network_contribution":0.0,"self_endowment_contribution":0.5570358100056463,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":40,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1649937,"name":"Christina Chrisman","orcid":null,"position":1,"is_corresponding":false},{"id":1681654,"name":"Drew Houston","orcid":null,"position":2,"is_corresponding":false},{"id":1681655,"name":"Clara Chow Haws","orcid":null,"position":3,"is_corresponding":false},{"id":863276,"name":"Alan Wang","orcid":"0000-0001-9749-0082","position":4,"is_corresponding":false},{"id":1681657,"name":"Suraj Muley","orcid":null,"position":5,"is_corresponding":false},{"id":1681652,"name":"Uzma Usman","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"The use of eculizumab in ventilator‐dependent myasthenia gravis patients","abstract":"<jats:title>Abstract</jats:title><jats:sec><jats:title>Introduction/Aims</jats:title><jats:p>Eculizumab has been shown to be efficacious in acetylcholine receptor antibody–positive (AChR<jats:sup>+</jats:sup>) Myasthenia Gravis Foundation of America (MGFA) class II, III, and IV generalized myasthenia gravis (gMG) patients. However, it has not been studied in MGFA class V gMG patients.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We report three AChR<jats:sup>+</jats:sup>, refractory, MGFA class V gMG patients treated with eculizumab. MGFA class, MG‐Composite (MGC) score and MG Activities of Daily Living (MG‐ADL) score were assessed before and after eculizumab.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Two of three gMG patients, refractory to intravenous immunoglobulin, plasmapheresis, prednisone, and (in one case) rituximab, showed a robust response to eculizumab with marked improvement in MGFA, MG‐ADL, and MGC measures. The third patient showed a partial response to eculizumab but remained on noninvasive ventilation and gastrostomy intubation. Patients 1 and 2 achieved minimal manifestation status at week 4 and week 6, respectively, and showed continued improvement on MG‐ADL and MGC scores through weeks 55 and 43, respectively, with eculizumab. The third patient showed a partial response at week 10, followed by a slight decline in his MG‐ADL score, but noted a slow but an incomplete improvement afterward on MG‐ADL and MGC scores, possibly due to delayed eculizumab infusion.</jats:p></jats:sec><jats:sec><jats:title>Discussion</jats:title><jats:p>Eculizumab may play a role in the treatment of patients with MGFA class V, refractory gMG. Larger studies are required to provide further evidence.</jats:p></jats:sec>","is_dataset_classified":null,"base_score":3.713572066704308,"endowment":3.713572066704308,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"34008175","pmcid":null,"openalex_id":"https://openalex.org/W3162111278","authors":[],"funders":[],"total_grants":0,"fwci":2.1789,"citation_percentile":0.89793861,"influential_citations":0,"citation_trend":[{"year":2021,"count":1},{"year":2022,"count":4},{"year":2023,"count":7},{"year":2024,"count":9},{"year":2025,"count":13},{"year":2026,"count":5}],"oa_status":"closed","license":"http://onlinelibrary.wiley.com/termsAndConditions#vor","oa_locations":[{"url":"https://onlinelibrary.wiley.com/doi/pdf/10.1002/mus.27326","host_type":"publisher"},{"url":"https://onlinelibrary.wiley.com/doi/full-xml/10.1002/mus.27326","host_type":"publisher"},{"url":"https://doi.org/10.1002/mus.27326","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/34008175","host_type":"repository"}],"fields_of_study":["Myasthenia Gravis and Thymoma","Adrenal Hormones and Disorders","Peripheral Neuropathies and Disorders","Activities of Daily Living","Adult","Aged","Antibodies, Monoclonal, Humanized","Complement Inactivating Agents","Female","Humans","Male","Middle Aged","Myasthenia Gravis","Rituximab","Ventilators, Mechanical"],"mesh_terms":["Rituximab","Activities of Daily Living","Adult","Aged","Female","Humans","Male","Middle Aged","Myasthenia Gravis","Ventilators, Mechanical","Complement Inactivating Agents","Antibodies, Monoclonal, Humanized"],"keywords":["Eculizumab","Medicine","Myasthenia gravis","Refractory (planetary science)","Plasmapheresis","Rituximab","Internal medicine","Gastroenterology","Antibody","Immunology","Complement system","Ventilator-dependent","Mgc, Mgfa"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-09T10:03:39.726213Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}