{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T01:38:20Z","timestamp":1789609100820,"version":"build-2803163510"},"reference-count":34,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2024,4,27]],"date-time":"2024-04-27T00:00:00Z","timestamp":1714176000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JPM"],"abstract":"<jats:p>Anti-signal recognition particle myopathy (anti-SRP myopathy) is a rare subtype of immune-mediated inflammatory myopathy characterized by muscle weakness and anti-SRP autoantibodies. Although plasma exchange (PE) is used in severe cases, its role remains unclear. A systematic review was conducted following PRISMA guidelines, identifying 23 patients with anti-SRP myopathy treated with PE. Data on demographics, clinical features, laboratory findings, treatments, and outcomes were analyzed combining individual patient data if available. Sixteen (69.6%) patients were male, with muscle weakness as the predominant symptom in 100% of cases. After PE, most patients showed improvement in symptoms, and the proportion of patients with muscle weakness was reduced (p = 0.001). Relapse occurred in 17.4% of the cases. The incidence of adverse events was low (8.7%). Despite limitations, including a small sample size and heterogeneous data, our systematic review suggests that PE may be effective in inducing remission and controlling symptoms in anti-SRP myopathy, particularly in severe cases. Since evidence on PE in anti-SRP myopathy is limited, further research, including prospective multicenter studies, is warranted to understand better its efficacy and safety and establish its role in treatment algorithms.<\/jats:p>","DOI":"10.3390\/jpm14050461","type":"journal-article","created":{"date-parts":[[2024,4,30]],"date-time":"2024-04-30T09:50:07Z","timestamp":1714470607000},"page":"461","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Plasma Exchange in Anti-Signal Recognition Particle Myopathy: A Systematic Review and Combined Analysis of Patient Individual Data"],"prefix":"10.3390","volume":"14","author":[{"given":"Pablo","family":"Mart\u00ednez-Rodr\u00edguez","sequence":"first","affiliation":[{"name":"Connective Tissue Diseases Unit, Department of Internal Medicine, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"},{"name":"Department of Medicine, Faculty of Medicine, University of Salamanca, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda","family":"Escribano-Iglesias","sequence":"additional","affiliation":[{"name":"Department of Radiology, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1ngel-P.","family":"Crisolino-Pozas","sequence":"additional","affiliation":[{"name":"Connective Tissue Diseases Unit, Department of Internal Medicine, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noelia","family":"Cubino-Boveda","sequence":"additional","affiliation":[{"name":"Connective Tissue Diseases Unit, Department of Internal Medicine, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"},{"name":"Department of Medicine, Faculty of Medicine, University of Salamanca, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2949-0627","authenticated-orcid":false,"given":"Miriam","family":"L\u00f3pez-Parra","sequence":"additional","affiliation":[{"name":"Department of Hematology, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1269-4487","authenticated-orcid":false,"given":"Miguel","family":"Marcos","sequence":"additional","affiliation":[{"name":"Connective Tissue Diseases Unit, Department of Internal Medicine, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"},{"name":"Department of Medicine, Faculty of Medicine, University of Salamanca, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2345-2873","authenticated-orcid":false,"given":"Antonio-J.","family":"Chamorro","sequence":"additional","affiliation":[{"name":"Connective Tissue Diseases Unit, Department of Internal Medicine, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain"},{"name":"Department of Medicine, Faculty of Medicine, University of Salamanca, 37001 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,4,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1038\/s41584-020-00515-9","article-title":"Immune-mediated necrotizing myopathy: Clinical features and pathogenesis","volume":"16","author":"Allenbach","year":"2020","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"102993","DOI":"10.1016\/j.autrev.2021.102993","article-title":"Immune-mediated necrotizing myopathy (IMNM): A myopathological challenge","volume":"21","author":"Merlonghi","year":"2022","journal-title":"Autoimmun. Rev."},{"key":"ref_3","unstructured":"Grau, J.M., Casademont, J., and Cardellach, F. (2016). Farreras Rozman Medicina Interna, Elservier. [18th ed.]."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s11926-018-0732-6","article-title":"Immune-Mediated Necrotizing Myopathy","volume":"20","author":"Mammen","year":"2018","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1097\/BOR.0b013e32834b324b","article-title":"Necrotizing autoimmune myopathy","volume":"23","author":"Liang","year":"2011","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1007\/s11926-015-0548-6","article-title":"Immune-Mediated Necrotizing Myopathy: Update on Diagnosis and Management","volume":"17","author":"Basharat","year":"2015","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_7","first-page":"655","article-title":"Peculiar clinicopathological features of immune-mediated necrotizing myopathies","volume":"30","author":"Allenbach","year":"2018","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_8","unstructured":"Loscalzo, J., Kasper, D., Fauci, A.S., Hauser, A., and Longo, D. (2022). Harrison: Principios de Medicina Interna, McGraw-Hill. [19th ed.]."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1136\/annrheumdis-2017-211468","article-title":"2017 European League Against Rheumatism\/American College of Rheumatology classification criteria for adults and juvenile idiopathic inflammatory myopathies and their major subgroups","volume":"76","author":"Lundberg","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.nmd.2017.09.016","article-title":"224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies","volume":"28","author":"Allenbach","year":"2018","journal-title":"Neuromuscul. Disord."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/s11940-018-0521-6","article-title":"Novel Therapeutic Options in Treatment of Idiopathic Inflammatory Myopathies","volume":"20","author":"Goyal","year":"2018","journal-title":"Curr. Treat. Options Neurol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1734","DOI":"10.1056\/NEJMra1402225","article-title":"Inflammatory Muscle Diseases","volume":"372","author":"Dalakas","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_13","unstructured":"Roger, A. (2024, April 22). Diagn\u00f3stico Electromiogr\u00e1fico de las Enfermedades Neuromusculares. Available online: http:\/\/scielo.sld.cu\/scielo.php?script=sci_arttext&pid=S0138-65572007000100008&lng=es."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1007\/s40674-021-00174-1","article-title":"Therapeutic Management of Immune-Mediated Necrotizing Myositis","volume":"7","author":"Weeding","year":"2021","journal-title":"Curr. Treatm. Opt. Rheumatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1016\/j.nmd.2006.03.002","article-title":"Marked efficacy of a therapeutic strategy associating prednisone and plasma exchange followed by rituximab in two patients with refractory myopathy associated with antibodies to the signal recognition particle (SRP)","volume":"16","author":"Arlet","year":"2006","journal-title":"Neuromuscul. Disord."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1016\/j.braindev.2012.02.009","article-title":"A pediatric patient with myopathy associated with antibodies to a signal recognition particle","volume":"34","author":"Kawabata","year":"2012","journal-title":"Brain Dev."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1328","DOI":"10.1002\/acr.20219","article-title":"Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: A case series","volume":"62","author":"Valiyil","year":"2010","journal-title":"Arthritis Care Res."},{"key":"ref_18","first-page":"434","article-title":"Antisignal recognition particle antibodies-related cardiomyopathy","volume":"127","author":"Terrier","year":"2013","journal-title":"Circulation"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1007\/s00296-009-1059-4","article-title":"Signal recognition particle (SRP) positive myositis in a patient with cryptogenic organizing pneumonia (COP)","volume":"30","author":"Wantke","year":"2010","journal-title":"Rheumatol. Int."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"529","DOI":"10.3109\/14397595.2013.852852","article-title":"Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide","volume":"24","author":"Momomura","year":"2014","journal-title":"Mod. Rheumatol."},{"key":"ref_21","first-page":"164","article-title":"Anti-SRP myopathy in 2 different ethnic groups","volume":"19","author":"Tiwana","year":"2018","journal-title":"J. Clin. Neuromuscul. Dis."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Kamo, H., Hatano, T., Kanai, K., Aoki, N., Kamiyama, D., Yokoyama, K., Takanashi, M., Yamashita, Y., Shimo, Y., and Hattori, N. (2019). Pembrolizumab-related systemic myositis involving ocular and hindneck muscles resembling myasthenic gravis: A case report. BMC Neurol., 19.","DOI":"10.1186\/s12883-019-1416-1"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1212\/WNL.94.15_supplement.1139","article-title":"Successful recovery of anti-SRP myopathy with subcutaneous methotrexate after 17 years of poor response to immunomodulation","volume":"94","author":"Harada","year":"2020","journal-title":"Neurology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2625","DOI":"10.1093\/rheumatology\/keab629","article-title":"Plasma exchange therapy in refractory inflammatory myopathy with anti-signal recognition particle antibody: A case series","volume":"61","author":"Zhang","year":"2022","journal-title":"Rheumatology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1016\/j.lpm.2012.01.006","article-title":"Myopathie n\u00e9crosante avec auto-anticorps anti-SRP: Efficacit\u00e9 transitoire d\u2019une strat\u00e9gie th\u00e9rapeutique associant \u00e9changes plasmatiques et rituximab","volume":"41","author":"Savey","year":"2012","journal-title":"Presse Med."},{"key":"ref_26","first-page":"e35471","article-title":"Marked Efficacy of a Therapeutic Strategy in a Patient With Necrotizing Myopathy Associated With Anti-signal Recognition Particle (SRP) Autoantibodies","volume":"15","author":"Almaghrabi","year":"2023","journal-title":"Cureus"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"17562864231154306","DOI":"10.1177\/17562864231154306","article-title":"Comparison of IVIg and TPE efficacy in the treatment of neurological disorders: A systematic literature review","volume":"16","author":"Pinto","year":"2023","journal-title":"Ther. Adv. Neurol. Disord."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Pavlekovics, M., Engh, M.A., Lugosi, K., Szabo, L., Hegyi, P., Terebessy, T., Csukly, G., Molnar, Z., Illes, Z., and Lovas, G. (2023). Plasma Exchange versus Intravenous Immunoglobulin in Worsening Myasthenia Gravis: A Systematic Review and Meta-Analysis with Special Attention to Faster Relapse Control. Biomedicines, 11.","DOI":"10.3390\/biomedicines11123180"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"103661","DOI":"10.1016\/j.transci.2023.103661","article-title":"Therapeutic plasma exchange-free treatment for first-episode TTP: A systematic review","volume":"62","author":"Wang","year":"2023","journal-title":"Transfus. Apher. Sci."},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Della-Marina, A., Pawlitzki, M., Ruck, T., van-Baalen, A., Vogt, N., Schweiger, B., Hertel, S., K\u00f6lbel, H., Wiendl, H., and Preu\u00dfe, C. (2021). Clinical course, myopathology and challenge of therapeutic intervention in pediatric patients with autoimmune-mediated necrotizing myopathy. Children, 8.","DOI":"10.3390\/children8090721"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"e96","DOI":"10.1212\/NXI.0000000000000096","article-title":"Clinical course and treatment of anti-HMGCR antibody-associated necrotizing autoimmune myopathy","volume":"2","author":"Ramanathan","year":"2015","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1136\/annrheumdis-2018-213518","article-title":"In vivo pathogenicity of IgG from patients with anti-SRP or anti-HMGCR autoantibodies in immune-mediated necrotising myopathy","volume":"78","author":"Bergua","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"219","DOI":"10.3899\/jrheum.220139","article-title":"Coexistence of Anti-Ro52 Antibodies in Anti-MDA5 Antibody\u2013Positive Dermatomyositis Is Highly Associated with Rapidly Progressive Interstitial Lung Disease and Mortality Risk","volume":"50","author":"Lv","year":"2023","journal-title":"J. Rheumatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"e002076","DOI":"10.1136\/bmjresp-2023-002076","article-title":"Association of anti-Ro52 autoantibody with interstitial lung disease in autoimmune diseases: A systematic review and meta-analysis","volume":"10","author":"Nayebirad","year":"2023","journal-title":"BMJ Open Respir. Res."}],"container-title":["Journal of Personalized Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4426\/14\/5\/461\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:34:30Z","timestamp":1760106870000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4426\/14\/5\/461"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,27]]},"references-count":34,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2024,5]]}},"alternative-id":["jpm14050461"],"URL":"https:\/\/doi.org\/10.3390\/jpm14050461","relation":{},"ISSN":["2075-4426"],"issn-type":[{"value":"2075-4426","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,27]]}}}