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Maintenance treatment with subcutaneous immunoglobulins in the long-term management of anti-HMCGR myopathy.
Neuromuscular Disorders ( IF 2.7 ) Pub Date : 2021-02-01 , DOI: 10.1016/j.nmd.2020.12.012
Angela Zuppa 1 , Chiara De Michelis 1 , Giuseppe Meo 1 , Valeria Prada 2 , Chiara Gemelli 1 , Maria Infantino 3 , Mariangela Manfredi 3 , Giampaola Pesce 4 , Alberto S Tagliafico 5 , Luana Benedetti 2 , Chiara Fiorillo 6 , Angelo Schenone 2 , Luca Quartuccio 7 , Marina Grandis 2
Affiliation  

We describe the clinical response to long-term subcutaneous immunoglobulins (SCIg) in anti-3‑hydroxy-3-methyl-glutaryl-coenzyme-A-reductase (anti-HMCGR) myopathy previously treated with intravenous immunoglobulins (IVIg). We collected data from patients affected by anti-HMGCR myopathy, switched from IVIg to SCIg therapy, after achieving clinical stabilization. The Medical Research Council sum score, creatine kinase (CK) levels, and anti-HMGCR antibodies were used to assess the response. We identified three patients with anti-HMGCR myopathy treated with SCIg with a favourable clinical course, allowing the maintenance of clinical stability, the reduction or suspension of steroids therapy and in two of them a complete CK normalization. Finally, anti-HMGCR antibodies tested in all patients after 12 months from SCIg starting, showed a global decrease. SCIg represent an useful alternative to long-term IVIg as already well known in several autoimmune neuromuscular disorders and inflammatory myopathies with advantages of lower side effects and home self-administration.

中文翻译:

在抗 HMCGR 肌病的长期管理中使用皮下免疫球蛋白进行维持治疗。

我们描述了先前用静脉内免疫球蛋白 (IVIg) 治疗的抗 3-羟基-3-甲基-戊二酰辅酶 A-还原酶 (抗 HMCGR) 肌病对长期皮下免疫球蛋白 (SCIg) 的临床反应。我们收集了受抗 HMGCR 肌病影响的患者的数据,在达到临床稳定后,从 IVIg 切换到 SCIg 治疗。医学研究委员会总分、肌酸激酶 (CK) 水平和抗 HMGCR 抗体用于评估反应。我们确定了三名接受 SCIg 治疗的抗 HMGCR 肌病患者具有良好的临床病程,允许维持临床稳定性,减少或暂停类固醇治疗,其中两名患者 CK 完全正常化。最后,从 SCIg 开始 12 个月后,在所有患者中测试的抗 HMGCR 抗体显示整体下降。
更新日期:2021-02-01
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