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Background: Subcutaneous immunoglobulin (SCIg) is a promising alternative to intravenous Ig (IVIg) for the treatment of idiopathic inflammatory myositis (IIM), thanks to its more favorable safety profile, reduced costs, and lower impact on patients' quality of life. We assessed the short- and long-term effectiveness and safety of recombinant human hyaluronidase-facilitated SCIg (hf-SCIg) in patients with IIM treated at different referral centers in Italy.
Methods: A multicenter, retrospective, real-life cohort study was conducted on consecutive adult patients diagnosed with IIM according to the EULAR/ACR criteria, treated with hf-SCIg for remission induction or maintenance. Hf-SCIg effectiveness was assessed in terms of variation in the Medical Research Council (MRC) score, serum creatine kinase (CK) values, clinical disease manifestations and daily prednisone dosage. Safety data were also collected.
Results: Twenty-six patients with IIM treated with hf-SCIg for remission induction (n = 5) or maintenance (n = 21) were included in the study (18 females; median age at diagnosis of 59 (IQR 42-64) years)). In most patients, hf-SCIg was started following previous IVIg treatment (23, 89 %) and was initiated in combination with oral corticosteroids (21, 81 %) and/or traditional or biologic DMARDs. Short-term use of hf-SCIg for remission induction appeared associated with a corticosteroid-sparing effect, without worsening of MRC score. Long-term hf-SCIg treatment for up to 24 months maintained clinical stability and serum CK levels with further improvement of MRC score. Hf-SCIg was well tolerated, with mild adverse events mostly related to local site reactions.
Conclusions: Hf-SCIg seems effective and safe for induce and maintain clinical remission in patients with IIM.
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http://dx.doi.org/10.1016/j.ejim.2025.05.016 | DOI Listing |
Clin Med (Lond)
September 2025
Rheumatology Research Group, Department of Inflammation and Ageing, College of Medicine & Health, University of Birmingham, Birmingham, UK.; National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
Immune-mediated inflammatory diseases (IMIDs) are a group of common clinically diverse conditions which are characterised by systemic inflammation. They often pose medical challenges due to their multi organ involvement, chronicity, associated co-morbidities and poor impact on quality of life to patients. The management for IMIDs has changed profoundly over the past twenty years with the paradigm of treatment shifting away from broad immunosuppression towards pathway specific targeted treatment.
View Article and Find Full Text PDFImmunol Invest
September 2025
Department of Dermatology, Hunan Key Laboratory of Medical Epigenomics, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China.
Objective: To examine whether features of the B cell receptor (BCR) IgG repertoire correlate with disease activity and clinical phenotypes in systemic autoimmune diseases (SAIDs).
Methods: High-throughput sequencing was performed on IgG heavy chain repertoires from 138 patients with SAIDs, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), scleroderma (SSc), and idiopathic inflammatory myopathy (IIM), as well as 36 healthy controls (HC). We analyzed V/D/J gene usage, clonal distribution and diversity, CDR3 length distribution and amino acid usage, and the correlation between specific BCR features and clinical features.
Int Immunopharmacol
September 2025
Department of Cuiying Biomedical Research Center, Lanzhou University Second Hospital, Lanzhou, China; Department of Rheumatology, Lanzhou University Second Hospital, Lanzhou, China; Gansu Province Clinical Research Center for Rheumatology, Lanzhou, Gansu, China. Electronic address:
Background: Previous studies by our research group have demonstrated that neutrophil extracellular traps (NETs) play a pathogenic role in myositis-associated interstitial lung disease (IIM-ILD). Based on this, we hypothesized that NETs may contribute to the pathogenesis of IIM-ILD by regulating macrophage polarization and pyroptosis.
Methods: Pathological studies were conducted using lung biopsy samples from a dermatomyositis-associated interstitial lung disease (DM-ILD) patient and lung tissues from experimental autoimmune myositis (EAM) mice.
Eur J Intern Med
August 2025
Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy; Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, Australia. Electronic address:
Background: Subcutaneous immunoglobulin (SCIg) is a promising alternative to intravenous Ig (IVIg) for the treatment of idiopathic inflammatory myositis (IIM), thanks to its more favorable safety profile, reduced costs, and lower impact on patients' quality of life. We assessed the short- and long-term effectiveness and safety of recombinant human hyaluronidase-facilitated SCIg (hf-SCIg) in patients with IIM treated at different referral centers in Italy.
Methods: A multicenter, retrospective, real-life cohort study was conducted on consecutive adult patients diagnosed with IIM according to the EULAR/ACR criteria, treated with hf-SCIg for remission induction or maintenance.
Front Immunol
September 2025
Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, United States.
Introduction: Vaccinations have had a transformative impact on public health, reducing the incidence of many infectious diseases and increasing survival. However, there remains uncertainty about the potential of vaccines to trigger autoimmune diseases such as the idiopathic inflammatory myopathies (IIM). Myositis after vaccination (MAV) is a rare clinical entity, but given immunogenetic associations with other adverse events, we explored genetic risk factors, particularly human leukocyte antigen (HLA) alleles and GM/KM immunoglobulin allotypes, that may predispose individuals to develop MAV.
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