Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal.
Unidade Local de Saúde de Santo António.
ARP Rheumatol. 2024 Jul-Sep;3(3):231-236. doi: 10.63032/ZVNO7794.
Anti-HMGCR myopathy is an increasingly recognized immune-mediated necrotizing myopathy. However, there are currently no evidence-based treatments available, so case reports and clinical experience are used to guide current management. We report a case of a 49-year-old man, treated with atorvastatin, who presented to the emergency department with progressive proximal muscle weakness. Anti-HMGCR antibodies were detected, and muscle biopsy revealed necrotizing myopathy. Initially, therapy with high-dose glucocorticoids and methotrexate was started, but 12 weeks later, the patient developed clinical deterioration with dysphagia. Then, he was successfully treated with one cycle of rituximab along with physical therapy. The use of rituximab in immune-mediated necrotizing myopathy has been heterogeneously described in the literature but mostly in case reports. The European Neuromuscular Centre working group recommends the use of rituximab in refractory cases. However, some studies highlight the importance of early and aggressive treatment for this disease. Clinical prospective studies are necessary to make proper evidence-based recommendations.
抗 HMGCR 肌病是一种日益被认识的免疫介导性坏死性肌病。然而，目前尚无循证治疗方法，因此采用病例报告和临床经验来指导当前的治疗。我们报告了一例 49 岁男性，因服用阿托伐他汀而发病，他因进行性近端肌无力而到急诊就诊。检测到抗 HMGCR 抗体，肌肉活检显示坏死性肌病。最初，开始使用大剂量糖皮质激素和甲氨蝶呤治疗，但 12 周后，患者出现吞咽困难的临床恶化。随后，他成功地接受了一个周期的利妥昔单抗联合物理治疗。利妥昔单抗在免疫介导性坏死性肌病中的应用在文献中有不同的描述，但大多是病例报告。欧洲神经肌肉中心工作组建议在难治性病例中使用利妥昔单抗。然而，一些研究强调了早期和积极治疗这种疾病的重要性。需要进行临床前瞻性研究，以便提出适当的循证建议。
