Efficacy and Safety of High-Dose Immunoglobulin-Based Regimen in Statin-Associated Autoimmune Myopathy: A Multi-Center and Multi-Disciplinary Retrospective Study
| Autor: | Paola Parronchi, Daniele Cammelli, Boaz Palterer, Elena Treppo, Marina Grandis, Mariangela Manfredi, Luca Quartuccio, Viviana Ravagnani, Salvatore De Vita, Martina Fabris, P. Tomietto, Maria Grazia Giudizi, Francesca Ligobbi, Maurizio Benucci, Maria Infantino |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Statin medicine.drug_class immune-mediated necrotizing myopathy anti-HMGCR antibody autoimmune myopathy statins lcsh:Medicine Muscle disorder Article 03 medical and health sciences 0302 clinical medicine Internal medicine Medicine Myopathy 030203 arthritis & rheumatology business.industry lcsh:R Antibody titer Autoantibody Retrospective cohort study General Medicine Regimen Methotrexate lipids (amino acids peptides and proteins) medicine.symptom business 030217 neurology & neurosurgery medicine.drug |
| Zdroj: | Journal of Clinical Medicine Volume 9 Issue 11 Journal of Clinical Medicine, Vol 9, Iss 3454, p 3454 (2020) |
| ISSN: | 2077-0383 |
| DOI: | 10.3390/jcm9113454 |
| Popis: | Statin-associated autoimmune myopathy is a rare muscle disorder, characterized by autoantibodies against HMGCR. The anti-HMGCR myopathy persists after statin, and often requires immunosuppressive therapy. However, there is not a standardized therapeutic approach. The purpose of this study is to report the effectiveness of the immunosuppressive treatment employed in a multi-center and multi-disciplinary cohort of patients affected by anti-HMGCR myopathy, in which an immunoglobulin (IVIG)-based treatment strategy was applied. We collected 16 consecutive patients with a diagnosis of anti-HMGCR myopathy, between 2012 and 2019, and recorded data on clinical and laboratory presentation (i.e., muscle strength, serum CK levels, and anti-HMGCR antibody titer) and treatment strategies. Our results highlight the safety and efficacy of an induction therapy combining IVIG with GCs and/or methotrexate to achieve persistent remission of the disease and steroid-free maintenance. Under IVIG-based regimens, clinical improvement and CK normalization occurred in more than two thirds of patients by six months. Relapse rate was low (3/16) and 2/3 relapses occurred after treatment suspension. Nearly 90% of the patients who successfully discontinued GCs were treated with a triple immunosuppressive regimen. In conclusion, an IVIG-based regimen, which particularly includes high-dose immunoglobulin, GCs and methotrexate, can provide a fast remission achievement with GC saving. |
| Databáze: | OpenAIRE |
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