Srinivasa Murthy Manasa, Haikal Ammar
Internal Medicine, Bayonne Medical Center, Bayonne, USA.
Rheumatology, Hackensack University Medical Center, Hackensack, USA.
Cureus. 2024 Aug 12;16(8):e66704. doi: 10.7759/cureus.66704. eCollection 2024 Aug.
We present a case of refractory cutaneous dermatomyositis (DM) in a 51-year-old Hispanic female which failed multiple treatments but found symptomatic relief with anifrolumab. Anifrolumab was the only treatment that was associated with significant improvement in the rash and pruritis of the patient and lowered her corticosteroid needs. To our knowledge, this is the only second case report that has shown success in treating refractory cutaneous symptoms of DM with anifrolumab after failing standard and multiple combinations of therapies. Anifrolumab is a new first-in-class human monoclonal antibody, which inhibits type 1 interferon receptor (IFN-1) and is used to treat systemic lupus erythematosus (SLE). It is FDA-approved for non-renal manifestations of SLE. This IFN pathway seems to be also active in patients with DM. The presence of IFN-1 and IFN-2 has been reported in muscle biopsies of patients with inflammatory myopathies. Moreover, the IFN activation signature is present in the muscle, blood, and skin of patients with DM. IFN-1 has been assumed to activate toll-like receptors which activate the dendritic cells leading to the secretion of cytokines and chemokines. This potential pathophysiological role of IFN in DM may explain the symptom improvement experienced by our patient after starting anifrolumab treatment. Anifrolumab has additionally been shown to have a good safety profile when used to treat patients with SLE with up to three years of treatment on background conventional disease-modifying antirheumatic drug (DMARD) therapies. In conclusion, SLE and DM share similarities in their pathophysiology and cutaneous disease involvement and can be differentiated clinically. Skin manifestations of DM can persist despite combinations of therapies even when weakness resolves. With this case report, we aim to highlight the possibility of utilizing anifrolumab for treating DM skin manifestations, especially in refractory cases. More research is needed to guide where anifrolumab stands in the therapeutic algorithm for DM. It is unknown whether it treats the myositis component, DM-related arthritis, or coexistent rheumatoid arthritis.
我们报告了一例51岁西班牙裔女性难治性皮肤型皮肌炎（DM）病例，该患者多种治疗均失败，但使用阿尼鲁单抗后症状得到缓解。阿尼鲁单抗是唯一一种使该患者皮疹和瘙痒症状显著改善并减少其皮质类固醇需求的治疗方法。据我们所知，这是第二例在标准治疗和多种联合治疗失败后使用阿尼鲁单抗成功治疗难治性皮肤型DM症状的病例报告。阿尼鲁单抗是一种新型的一流人类单克隆抗体，可抑制1型干扰素受体（IFN-1），用于治疗系统性红斑狼疮（SLE）。它已获美国食品药品监督管理局（FDA）批准用于SLE的非肾脏表现。该IFN途径在DM患者中似乎也有活性。在炎性肌病患者的肌肉活检中已报告有IFN-1和IFN-2存在。此外，DM患者的肌肉、血液和皮肤中存在IFN激活特征。IFN-1被认为可激活Toll样受体，从而激活树突状细胞，导致细胞因子和趋化因子的分泌。IFN在DM中的这种潜在病理生理作用可能解释了我们的患者在开始阿尼鲁单抗治疗后症状改善的原因。在背景常规改善病情抗风湿药物（DMARD）治疗的基础上，阿尼鲁单抗用于治疗SLE患者长达三年时，还显示出良好的安全性。总之，SLE和DM在病理生理和皮肤疾病受累方面有相似之处，且可在临床上进行区分。即使肌无力症状缓解，联合治疗后DM的皮肤表现仍可能持续。通过本病例报告，我们旨在强调使用阿尼鲁单抗治疗DM皮肤表现的可能性，尤其是在难治性病例中。需要更多研究来指导阿尼鲁单抗在DM治疗方案中的地位。目前尚不清楚它是否能治疗肌炎成分、DM相关关节炎或并存的类风湿关节炎。
