Letter to the Editor
Role of plasma exchange in myasthenia gravis in the shortage of immunoglublins
Papel del recambio plasmástico en el abordaje de la miastenia gravis ante el desabastecimiento de inmunoglobulinas
a Servicio de Hematología, Hospital de Barbastro. Barbastro, Huesca, Spain
b Banco de Sangre y Tejidos de Navarra, Pamplona, Spain
c Grupo Español de Aféresis (GEA); Aragón Health Research Institute (IACS), Aragon Institute for Health Research (IIS Aragon), Zaragoza, Spain
d Grupo Español de Rehabilitación Multimodal (GERM); Grupo de Investigación Gestión en el Paciente Sangrante-PBM, Instituto de Investigación Sanitaria, Hospital Universitaria La Paz (IdiPAZ), Madrid, Spain
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Barbastro, Huesca, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Banco de Sangre y Tejidos de Navarra, Pamplona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Grupo Español de Aféresis (GEA); Aragón Health Research Institute (IACS), Aragon Institute for Health Research (IIS Aragon), Zaragoza, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Grupo Español de Rehabilitación Multimodal (GERM); Grupo de Investigación Gestión en el Paciente Sangrante-PBM, Instituto de Investigación Sanitaria, Hospital Universitaria La Paz (IdiPAZ), Madrid, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Papel del recambio plasmástico en el abordaje de la miastenia gravis ante el desabastecimiento de inmunoglobulinas" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleDisplayedQuote" id="dsq0005"><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Pride (In The Name Of Love)</p><p id="spar0021" class="elsevierStyleSimplePara elsevierViewall">U2</p></span></p><p id="par0001" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Dear Editor,</span></p><p id="par0010" class="elsevierStylePara elsevierViewall">We have read a recent review on myasthenia gravis (MG) published in your journal. In the treatment section it is stated that “Both plasmapheresis and intravenous human immunoglobulins (IVIG) are indicated in patients with MG who have suffered an acute worsening and require a rapid therapeutic response. The efficacy of both treatments is similar, and the choice is based primarily on the patient's comorbidities, the availability of either treatment and the practitioner's experience with these treatments.”<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> However, we would like to make a few clarifications or observations.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Plasmapheresis would be the process, usually for donation purposes, of using cell separators to remove a small amount of plasma to obtain blood products such as albumin, immunoglobulins and clotting factors. Therapeutic plasma exchange (TPE), a type of therapeutic apheresis, involves the removal of plasma from the patient and its substitution with replacement solutions, which may be albumin, fresh frozen plasma and/or combinations of albumin with crystalloids or colloids, to maintain the patient in an euvolemic state for therapeutic purposes.</p><p id="par0020" class="elsevierStylePara elsevierViewall">According to the international consensus on MG treatment,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> TPE and IVIG are the short-term treatments of choice for acute, life-threatening attacks of respiratory failure or dysphagia in patients with refractory MG or prior to surgery for bulbar dysfunction. Both treatments have similar efficacy, with TPE being more effective in muscle-specific tyrosine kinase myasthenia gravis MuSK-MG cases, with TPE acting faster than IVIG in acute attacks.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The latest American Society for Apheresis (ASFA)<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Guidelines recommend with an evidence level 1B (category I) the use of TPE for moderate to severe MG (acute attacks, instability or resistance) and unstable disease prior to thymectomy.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Recently, the Navarra Health Service-Osasunbidea has published a report on the use of albumin and another report on the efficacy and safety of IVIG in neurological and haematological pathologies<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> which indicates that treatment of MG should be carried out with 4% albumin TPE and not with plasma (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). In chronic MG, evidence from RCTs is insufficient to determine whether IVIG is effective. There is still very little evidence on the efficacy of subcutaneous Ig in the treatment of MG, and no comparative studies are available against IVIG or other alternatives.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Although there is no definitive consensus on the therapeutic regimen, 3–6 sessions daily or every 48 h for 10−14 days are recommended for acute attacks, relapses or unstable disease. The duration of these will depend on the clinical scenario. The treatment regimen that has been shown to be most effective is to process 1–1.5 of the patient’s blood volume (68−72 ml/kg) at each session, with a recommended initial frequency of 3–5 procedures per week, for a period of one to three weeks.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Of no less significance is the economic impact of TPE vs IVIG in neurological pathology,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> which has recently been reviewed, with a clear benefit in direct costs and a high safety profile. It is important to remember the difficulties associated with IVIG supply, with the external dependence on paid donor plasma from the United States and the current inability of our country to produce barely a third of the IVIG consumed. This is why we consider it essential to make optimal and evidence-based use of this scarce human resource, and to have apheresis units available 24/7 so that emergency treatment can be initiated and instituted within 24 h of the indication being established.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0045" class="elsevierStylePara elsevierViewall">None. Article made on its own initiative, without external influence.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">None direct. Dr. García Erce has received scholarships, grants and funding to participate or attend courses, symposia, congresses from Terumo, Haemonetics, CSL, Octapharma. No conflict with Grifols.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Funding" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflict of interest" ] 2 => array:2 [ "identificador" => "xack745975" "titulo" => "Acknowledgements" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:3 [ "etiqueta" => "◊" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">These authors share first authorship.</p>" "identificador" => "fn0005" ] ] "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleSmallCaps">Note</span>: If IVIG is used: low doses (1 g/kg).</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Overall, the evidence shows that IVIGs have an adequate safety profile, although they should be avoided or used with caution in patients at high thromboembolic risk.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">IVIG: intravenous immunoglobulin; MAPAC: Spanish acronym for Improving the Appropriateness of Care and Clinical Practice (Initiative); TPE: therapeutic plasma exchange.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Entity \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">1st line \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">2nd line \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">3rd line \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Myasthenic exacerbation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Glucocorticoid ± immunosuppressant \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1st option: TPE \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If no response to glucocorticoids, immunosuppressants, TPEs or IVIG, and if Ab positive for acetylcholine receptors: add eculizumab, efgartigimod<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If TPE is not possible: IVIG is an option \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Myasthenic crisis (MGFA V) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">TPE + glucocorticoids (add or adjust dose if already taking) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If TPE is not possible or contraindicated: IVIG \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">– \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3542913.png" ] ] ] "notaPie" => array:1 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Currently not marketed in Spain (obtained through Medicamentos Extranjeros).</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">MAPAC group proposals on positioning the use of IVIGs.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Miastenia gravis. Actualización diagnóstica y terapéutica" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "D.A. García Estévez" 1 => "J. Pardo Fernández" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.medcli.2023.04.006" "Revista" => array:6 [ "tituloSerie" => "Med Clín (Barc)" "fecha" => "2023" "volumen" => "161" "paginaInicial" => "119" "paginaFinal" => "127" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/37248131" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "International consensus guidance for management of myasthenia gravis executive summary" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "D.B. Sanders" 1 => "G.I. Wolfe" 2 => "M. Benatar" 3 => "A. Evoli" 4 => "N.E. Gilhus" 5 => "I. Illa" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Neurology" "fecha" => "2016" "volumen" => "87" "paginaInicial" => "419" "paginaFinal" => "425" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Guidelines on the use of therapeutic apheresis in clinical practice – evidence-based approach from the writing committee of the American Society for Apheresis: the ninth special issue" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Connelly-Smith" 1 => "C.R. Alquist" 2 => "N.A. Aqui" 3 => "J.C. Hofmann" 4 => "R. Klingel" 5 => "O.A. Onwuemene" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1002/jca.22043" "Revista" => array:6 [ "tituloSerie" => "J Clin Apher" "fecha" => "2023" "volumen" => "38" "paginaInicial" => "77" "paginaFinal" => "278" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/37017433" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:1 [ "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "L. Leache" 1 => "M. Gutiérrez" 2 => "L.C. Saiz" 3 => "J. Erviti" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Libro" => array:2 [ "fecha" => "2023" "editorial" => "Servicio Navarro de Salud-Osasunbidea" ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Análisis de costes del tratamiento con recambio plasmático terapéutico frente al tratamiento con Inmunoglobulinas endovenosas en pacientes con enfermedades neurológicas de base inmune. Propuesta de optimización del uso de hemoderivados plasmáticos" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M.L. Antelo Caamaño" 1 => "S. Zalba Marcos" 2 => "M. Sarobe Carricas" 3 => "L. Torne Hernández" 4 => "E. Erro Aguirre" 5 => "J.A. 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Leire Leache and Marta Gutiérrez from MAPAC of the Navarrese Health Service-Osasunbidea.</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000016200000009/v2_202405200151/S2387020624001505/v2_202405200151/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000016200000009/v2_202405200151/S2387020624001505/v2_202405200151/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020624001505?idApp=UINPBA00004N" ]
