{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T23:20:49Z","timestamp":1768087249190,"version":"3.49.0"},"reference-count":53,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T00:00:00Z","timestamp":1642377600000},"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:sec><jats:title>Background<\/jats:title><jats:p>Immunoglobulin (Ig) replacement therapy represents a life-saving treatment in primary antibody deficiencies. The introduction of subcutaneous Ig (SCIg) administration brings a major improvement in quality of life for patients, compared to the traditional intravenous administration. In recent years, an additional role has been proposed for Ig therapy for various inflammatory and immune-mediated diseases. Consequently, the use of SCIg has expanded from immunodeficiencies to immune-mediated diseases, such as polymyositis (PM) and dermatomyositis (DM). Given the rarity of these conditions, it is still difficult to evaluate the real impact of SCIg treatment on PM and DM, and additional data are constantly required on this topic, particularly for long-term treatments in real-life settings.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>This study aimed to increase the knowledge about the anti-inflammatory and immunomodulatory effects of SCIg treatment for myositis. To this aim, a long-term evaluation of the effectiveness of 20% human SCIg treatment (20% SCIg, Hizentra<jats:sup>\u00ae<\/jats:sup>, CSL Behring) was carried out in patients with PM\/DM in care at our Center. In addition, an evaluation of the 20% SCIg therapy in CVID patients was provided. This analysis, beside adding knowledge about the use of SCIg therapy in this real-life setting, was intended as a term of comparison, regarding the safety profile.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Results support the beneficial effect and tolerability of long-term 20% SCIg therapy in PM\/DM patients, reporting a significant improvement in creatine kinase levels, muscle strength, skin conditions, dysphagia, disease activity (MITAX score) and disability (HAQ-DI score). None of the patients reported systemic reactions. The duration of the reported local reactions was a few hours in 80% of the patients, and all resolved spontaneously. CVID patients reported an improvement in all the considered effectiveness parameters at the end of 20% SCIg therapy. The frequency of the adverse events reported by PM\/DM patients was not different from what reported in CVID patients, where the use of SCIg therapy is more consolidated<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study suggests that 20% SCIg treatment represents a viable and safe treatment for PM\/DM patients and a valid therapeutic alternative to IVIg, with important advantages for patients\u2019 quality of life.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2021.805705","type":"journal-article","created":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T06:49:47Z","timestamp":1642402187000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":10,"title":["Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients"],"prefix":"10.3389","volume":"12","author":[{"given":"Maria Giovanna","family":"Danieli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacopo Umberto","family":"Verga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Mezzanotte","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irene","family":"Terrenato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia","family":"Svegliati","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Beatrice","family":"Bilo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianluca","family":"Moroncini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2022,1,17]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"S1","DOI":"10.1016\/j.jaci.2016.09.023","article-title":"Update on the Use of Immunoglobulin in Human Disease: A Review of Evidence","volume":"139","author":"Perez","year":"2017","journal-title":"J\u00a0Allergy Clin Immunol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2014.00629","article-title":"Is Dosing of Therapeutic Immunoglobulins Optimal? A Review of a Three-Decade Long Debate in Europe","volume":"5","author":"Kerr","year":"2014","journal-title":"Front Immunol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-011-9511-0","article-title":"Effectiveness of Immunoglobulin Replacement Therapy on Clinical Outcome in Patients With Primary Antibody Deficiencies: Results From a Multicenter Prospective Cohort Study","volume":"31","author":"Quinti","year":"2011","journal-title":"J Clin Immunol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.iac.2008.01.008","article-title":"Principles of and Advances in Immunoglobulin Replacement Therapy for Primary Immunodeficiency","volume":"28","author":"Berger","year":"2008","journal-title":"Immunol Allergy Clin N Am"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.634181","article-title":"Update on Infections in Primary Antibody Deficiencies","volume":"12","author":"Demirdag","year":"2021","journal-title":"Front Immunol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-016-0243-z","article-title":"Quantitative Evidence of Wear-Off Effect at the End of the Intravenous IgG (IVIG) Dosing Cycle in Primary Immunodeficiency","volume":"36","author":"Rojavin","year":"2016","journal-title":"J\u00a0Clin Immunol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1007\/s40261-015-0270-1","article-title":"Subcutaneous Immunoglobulin Replacement Therapy in Patients With Primary Immunodeficiency in Routine Clinical Practice: The VISPO Prospective Multicenter Study","volume":"35","author":"Vultaggio","year":"2015","journal-title":"Clin Drug Investig"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2013.10.008","article-title":"Long-Term Efficacy, Safety, and Tolerability of Hizentra\u00ae for Treatment of Primary Immunodeficiency Disease","volume":"150","author":"Jolles","year":"2014","journal-title":"Clin Immunol"},{"key":"B9","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1016\/j.clim.2011.06.002","article-title":"Efficacy and Safety of Hizentra in Patients With Primary Immunodeficiency After a Dose-Equivalent Switch From Intravenous or Subcutaneous Replacement Therapy","volume":"141","author":"Jolles","year":"2011","journal-title":"Clin Immunol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.2165\/11587030-000000000-00000","article-title":"Pharmacokinetics of Subcutaneous IgPro20 in Patients With Primary Immunodeficiency","volume":"50","author":"Wasserman","year":"2011","journal-title":"Clin Pharmacokinet"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2009.04027.x","article-title":"Subcutaneous Immunoglobulin: Opportunities and Outlook","volume":"158","author":"Misbah","year":"2009","journal-title":"Clin Exp Immunol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1023\/A:1006678312925","article-title":"The Comparison of the Efficacy and Safety of Intravenous Versus Subcutaneous Immunoglobulin Replacement Therapy","volume":"20","author":"Chapel","year":"2000","journal-title":"J Clin Immunol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"100068","DOI":"10.1016\/j.waojou.2019","article-title":"Impact of IVIG vs. SCIG on IgG Trough Level and Infection Incidence in Primary Immunodeficiency Diseases: A Systematic Review and Meta-Analysis of Clinical Studies","volume":"12","author":"Shrestha","year":"2019","journal-title":"World Allergy Organ J"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2014.00690","article-title":"Mechanisms of Action of Ig Preparations: Immunomodulatory and Anti-Inflammatory Effects","volume":"5","author":"Matucci","year":"2015","journal-title":"Front Immunol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1503\/cmaj.130375","article-title":"Intravenous Immunoglobulin as Clinical Immune-Modulating Therapy","volume":"187","author":"Gilardin","year":"2015","journal-title":"CMAJ"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.666879","article-title":"Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies","volume":"12","author":"Farini","year":"2021","journal-title":"Front Immunol"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2019.102426","article-title":"Subcutaneous Immunoglobulin in Inflammatory Myopathies: Efficacy in Different Organ Systems","volume":"19","author":"Danieli","year":"2020","journal-title":"Autoimmun Rev"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.08.023","article-title":"Impact of Treatment on Survival in Polymyositis and Dermatomyositis. A Single-Centre Long-Term Follow-Up Study","volume":"13","author":"Danieli","year":"2014","journal-title":"Autoimmun Rev"},{"key":"B19","doi-asserted-by":"publisher","first-page":"1993","DOI":"10.1056\/NEJM199312303292704","article-title":"A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis","volume":"329","author":"Dalakas","year":"1993","journal-title":"N Engl J Med"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-211868","article-title":"The EuroMyositis Registry: An International Collaborative Tool to Facilitate Myositis Research","volume":"77","author":"Lilleker","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2018.41","article-title":"Classification of Myositis","volume":"14","author":"Lundberg","year":"2018","journal-title":"Nat Rev Rheumatol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1402225","article-title":"Inflammatory Muscle Diseases","volume":"372","author":"Dalakas","year":"2015","journal-title":"N Engl J Med"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtauto.2019.100018","article-title":"Polymyositis and Dermatomyositis - Challenges in Diagnosis and Management","volume":"2","author":"Yang","year":"2019","journal-title":"J Transl Autoimmun"},{"key":"B24","doi-asserted-by":"publisher","first-page":"87","DOI":"10.1007\/s13317-014-0063-1","article-title":"Assessment of Patients With Idiopathic Inflammatory Myopathies and Isolated Creatin-Kinase Elevation","volume":"5","author":"Iaccarino","year":"2014","journal-title":"Autoimmun Highlights"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410370709","article-title":"Immunopathogenesis of Inflammatory Myopathies","author":"Dalakas","year":"1995","journal-title":"Ann Neurol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.2217\/imt.10.108","article-title":"L-Proline-Stabilized Human IgG: Privigen\u00ae 10% for Intravenous Use and Hizentra\u00ae 20% for Subcutaneous Use","volume":"3","author":"Berger","year":"2011","journal-title":"Immunotherapy"},{"key":"B27","unstructured":""},{"key":"B28","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1177\/0394632016681577","article-title":"Shift From Intravenous or 16% Subcutaneous Replacement Therapy to 20% Subcutaneous Immunoglobulin in Patients With Primary Antibody Deficiencies","volume":"30","author":"Canessa","year":"2017","journal-title":"Int J Immunopathol Pharmacol"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-211468","article-title":"2017 European League Against Rheumatism\/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups","volume":"76","author":"Lundberg","year":"2017","journal-title":"Ann Rheum Dis"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2010.09.004","article-title":"Subcutaneous Immunoglobulin in Polymyositis and Dermatomyositis: A Novel Application","volume":"10","author":"Danieli","year":"2011","journal-title":"Autoimmun Rev"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20532","article-title":"Measures of Adult and Juvenile Dermatomyositis, Polymyositis, and Inclusion Body Myositis: Physician and Patient\/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)\/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), Physician Global Damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI)","author":"Rider","year":"2011","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"B32","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1093\/rheumatology\/keg427","article-title":"International Myositis and Clinical Studies Group (IMACS). International Consensus Outcome Measures for Patients With Idiopathic Inflammatory Myopathies. Development and Initial Validation of Myositis Activity and Damage Indices in Patients With Adult Onset Disease","volume":"43","author":"Isenberg","year":"2004","journal-title":"Rheumatol (Oxf)"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.142117","article-title":"Interrater Reliability and Aspects of Validity of the Myositis Damage Index","volume":"70","author":"Sultan","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"B34","unstructured":""},{"key":"B35","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1016\/j.jaip.2015.07.025","article-title":"International Consensus Document (ICON): Common Variable Immunodeficiency Disorders","volume":"4","author":"Bonilla","year":"2016","journal-title":"J Allergy Clin Immunol Pract"},{"key":"B36","unstructured":"Common Variable Immunodeficiency Diagnostic Criteria"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-124545","article-title":"Common Variable Immunodeficiency Disorders: Division Into Distinct Clinical Phenotypes","volume":"112","author":"Chapel","year":"2008","journal-title":"Blood"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1159\/000441490","article-title":"Subcutaneous Immunoglobulin Use in Inclusion Body Myositis: A Review of 6 Cases","volume":"7","author":"Cherin","year":"2015","journal-title":"Case Rep Neurol"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1111\/trf.14427","article-title":"Clinical Indications for Intravenous Immunoglobulin Utilization in a Tertiary Medical Center: A 9-Year Retrospective Study","volume":"58","author":"Shemer","year":"2018","journal-title":"Transfusion"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2012.08.017","article-title":"Tregitope Update: Mechanism of Action Parallels IVIg","volume":"12","author":"Cousens","year":"2013","journal-title":"Autoimmun Rev"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-011-8278-7","article-title":"Long-Term Therapy With Intravenous Immunoglobulin Is Beneficial in Patients With Autoimmune Diseases","volume":"42","author":"Zandman-Goddard","year":"2012","journal-title":"Clin Rev Allergy Immunol"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.08.018","article-title":"Subcutaneous IgG in Immune-Mediate Diseases: Proposed Mechanisms of Action and Literature Review","volume":"13","author":"Danieli","year":"2014","journal-title":"Autoimmun Rev"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.12.003","article-title":"Long-Term Subcutaneous Immunoglobulin Use in Inflammatory Myopathies: A Retrospective Review of 19 Cases","volume":"15","author":"Cherin","year":"2016","journal-title":"Autoimmun Rev"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1002\/cpdd.753","article-title":"Pharmacokinetic Analysis of Weekly Versus Biweekly Igpro20 Dosing in Patients With Primary Immunodeficiency","volume":"9","author":"Rojavin","year":"2020","journal-title":"Clin Pharmacol Drug Dev"},{"key":"B45","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1007\/s13554-014-0018-0","article-title":"Enhancing Patient Flexibility of Subcutaneous Immunoglobulin G Dosing: Pharmacokinetic Outcomes of Various Maintenance and Loading Regimens in the Treatment of Primary Immunodeficiency","volume":"4","author":"Sidhu","year":"2014","journal-title":"Biol Ther"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-018-0560-5","article-title":"Long-Term Efficacy and Safety of Hizentra\u00ae in Patients With Primary Immunodeficiency in Japan, Europe, and the United States: A Review of 7 Phase 3 Trials","volume":"38","author":"Jolles","year":"2018","journal-title":"J Clin Immunol"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.15761\/CCRR.1000149","article-title":"Improved Outcomes on Subcutaneous IgG in Patients With Humoral Immunodeficiency and Co-Morbid Bowel Disease","volume":"1","author":"Shah","year":"2015","journal-title":"Clin Case Rep Rev"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.2217\/imt.16.9","article-title":"The Potential of Intravenous Immunoglobulins for Cancer Therapy: A Road That is Worth Taking","volume":"8","author":"Corb\u00ed","year":"2016","journal-title":"Immunotherapy"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1385\/CRIAI:29:3:307","article-title":"Uncovering the Hidden Potential of Intravenous Immunoglobulin as an Anticancer Therapy","volume":"29","author":"Sapir","year":"2005","journal-title":"Clin Rev Allergy Immunol"},{"key":"B50","article-title":"Potential Anti-Tumor Activity of Intravenous and Subcutaneous Immunoglobulin","volume":"20","author":"Danieli","year":"2018","journal-title":"Isr Med Assoc J"},{"key":"B51","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/j.autrev.2015.09.002","article-title":"Management of Adverse Events in the Treatment of Patients With Immunoglobulin Therapy: A Review of Evidence","volume":"15","author":"Cherin","year":"2016","journal-title":"Autoimmun Rev"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-007-9169-9","article-title":"Prospective Study on CVID Patients With Adverse Reactions to Intravenous or Subcutaneous IgG Administration","volume":"28","author":"Quinti","year":"2008","journal-title":"J Clin Immunol"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-013-2478-x","article-title":"Open-Label Study on Treatment With 20% Subcutaneous IgG Administration in Polymyositis and Dermatomyositis","volume":"33","author":"Danieli","year":"2014","journal-title":"Clin Rheumatol"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2021.805705\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T06:49:53Z","timestamp":1642402193000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2021.805705\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,17]]},"references-count":53,"alternative-id":["10.3389\/fimmu.2021.805705"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2021.805705","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,17]]},"article-number":"805705"}}