Pinheiro Guiomar, Costa Ana Rita, Campar Ana, Mendonça Teresa
Internal Medicine Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Department of Clinical Immunology, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Eur J Case Rep Intern Med. 2020 Mar 2;7(4):001485. doi: 10.12890/2020_001485. eCollection 2020.
Fasciitis with eosinophilia (FE) is a rare connective tissue disease. Due to its rarity, large-scale studies are lacking, which makes its treatment challenging. Systemic corticosteroids (SCSs) are the cornerstone of treatment; however, additional immunosuppressive drugs (ISDs) are frequently necessary (usually methotrexate). We report 2 patients, for whom an SCS and methotrexate were not a viable long-term option. In the first case, we were unable to taper the SCS dose without symptom relapse, the patient showed only a partial response to methotrexate and presented side effects. The second case never fully responded to the SCS and methotrexate and demonstrated serious SCS adverse effects. Both patients were started on tocilizumab with extremely favourable results, making this drug a potential therapeutic weapon for these patients.
The treatment of FE is challenging and mainly based on retrospective reviews, open-label trials and case reports, all of which included a small number of patients.Currently, systemic corticosteroids are the mainstay of treatment; however, other ISDs are frequently necessary.Cases showing a favourable clinical response to tocilizumab have recently been described in patients with corticosteroid-refractory disease, suggesting that this drug may potentially become a therapeutic weapon for these patients.
嗜酸性筋膜炎(FE)是一种罕见的结缔组织病。由于其罕见性,缺乏大规模研究,这使得其治疗具有挑战性。全身用糖皮质激素(SCSs)是治疗的基石;然而,通常还需要加用其他免疫抑制药物(ISDs)(通常是甲氨蝶呤)。我们报告了2例患者,对于他们来说,SCS和甲氨蝶呤并非可行的长期治疗选择。在第一例中,我们无法在不减量SCS剂量的情况下避免症状复发,患者对甲氨蝶呤仅表现出部分反应并出现了副作用。第二例患者对SCS和甲氨蝶呤从未完全反应,并出现了严重的SCS不良反应。两名患者均开始使用托珠单抗,结果非常理想,使这种药物成为这些患者潜在的治疗武器。
FE的治疗具有挑战性,主要基于回顾性综述、开放标签试验和病例报告,所有这些研究纳入的患者数量都很少。目前,全身用糖皮质激素是主要治疗方法;然而,通常还需要其他ISDs。最近有病例报告显示,托珠单抗对糖皮质激素难治性疾病患者有良好的临床反应,这表明这种药物可能成为这些患者的治疗武器。