Scleroderma-like disorders

硬皮病样疾病
  • 文章类型: Case Reports
    嗜酸性筋膜炎是一种病因不明的罕见结缔组织病。治疗选择包括大剂量皮质类固醇和其他免疫抑制药物。我们提出了一个典型的嗜酸性筋膜炎病例,对一线治疗没有反应,但英夫利昔单抗给药后明显改善。本报告表明,在最初治疗失败的情况下,英夫利昔单抗可能是一种相对安全有效的嗜酸性筋膜炎治疗方法.
    Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. This report demonstrates that in case of initial treatment failure, infliximab might be a relatively safe and effective way of eosinophilic fasciitis management.
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  • 文章类型: Case Reports
    Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue of unknown aetiology and poorly understood pathogenesis. We present the case of a 60-year-old man diagnosed with eosinophilic fasciitis with extensive cutaneous involvement and severe functional repercussion, which appeared weeks after suffering from pneumonia due to Legionella pneumophila. The patient did not experience any clinical response with high-dose corticosteroids, subcutaneous methotrexate, and intravenous immunoglobulins. Consequently, tocilizumab was initiated at 8 mg/Kg monthly achieving clinical response measured by a control MRI at the fifth dose. Response in terms of cutaneous thickness has been slower however favourable, therefore, more months of follow-up are necessary to assess the complete remission at skin level. EF treatment still constitutes a challenge, and experience with tocilizumab in the management of the disease is very limited. Through a systematic search of medical literature, we retrieved two cases describing EF treated with tocilizumab and several cases using another monoclonal antibody or Janus kinase inhibitor. We report the third case to our knowledge of the efficacy of tocilizumab in a refractory EF to corticosteroids and other immunosuppressive drugs.
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