荨麻疹，发烧和关节炎：难治性成人发作的斯蒂尔氏病，对妥珠单抗反应良好。,Dermatologic Therapy

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荨麻疹，发烧和关节炎：难治性成人发作的斯蒂尔氏病，对妥珠单抗反应良好。
Dermatologic Therapy ( IF 3.7 ) Pub Date : 2019-08-12 , DOI: 10.1111/dth.13041
Bartlomiej Wawrzycki ₁ , Dorota Krasowska ₁ , Aldona Pietrzak ₁ , Ewa Wielosz ₂ , Maria Majdan ₂ , Torello Lotti ₃

Affiliation

成人性斯蒂尔氏病（AOSD）是一种病因尚未完全了解的罕见全身性炎症性疾病。先天免疫系统的异常激活和几种促炎介质的过度产生被认为是疾病发病机理中的关键组成部分。由于广泛的鉴别诊断和没有特定的生物标志物，AOSD仍然构成挑战。四个主要症状是：quotidian尖峰热，关节受累，e逝的鲑鱼皮疹，皮疹和嗜中性白血球增多。我们介绍了一例61岁女性，复发性荨麻疹皮疹，伴有高烧，关节嫩，喉咙痛，肝脏肿大，炎症反应物升高和高铁蛋白血症的发作。经过大量检查后，患者符合AOSD的标准。她对糖皮质激素和改变疾病的抗风湿药（DMARDs）无效。最后，在通过传统DMAR的Tocilizumab（TCZ）给药实现疾病控制的几次失败尝试之后，人源化的抗IL-6受体拮抗剂显着改善了疾病。由于皮肤表现是AOSD的普遍特征，因此应作为皮肤病变和体质症状患者的鉴别诊断之一。生物药物代表了对传统疗法难治的AOSD患者的重要治疗进展。人源化的抗IL-6受体拮抗剂可显着改善疾病。由于皮肤表现是AOSD的普遍特征，因此应作为皮肤病变和体质症状患者的鉴别诊断之一。生物药物代表了对传统疗法难治的AOSD患者的重要治疗进展。人源化的抗IL-6受体拮抗剂可显着改善疾病。由于皮肤表现是AOSD的共同特征，因此应作为皮肤病变和体质症状患者的鉴别诊断之一。生物药物代表了对传统疗法难治的AOSD患者的重要治疗进展。

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Urticarial rash, fever, and arthritis: A case of refractory Adult-onset Still's disease with good response to tocilizumab.

Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disorder of not completely understood etiology. Aberrant activation of the innate immune system and overproduction of several pro-inflammatory mediators are considered a critical component in disease pathogenesis. AOSD still poses a challenge due to the broad range of differential diagnoses and no specific biomarkers. Four cardinal symptoms are quotidian spiking fever, joint involvement, evanescent salmon pink-rash rash, and leukocytosis with neutrophilia. We present a case of a 61-year-old female with a recurrent urticarial rash accompanied by attacks of high fever, tender joints, sore throat, enlarged liver, elevated inflammatory reactants, and hyperferritinemia. After an extensive workup, the patient fulfilled the criteria of AOSD. She was refractory to the glucocorticosteroids and disease-modifying anti-rheumatic drugs (DMARDs). Finally, after several unsuccessful attempts to achieve disease control with traditional DMAR's administration of Tocilizumab (TCZ), a humanized anti-IL-6 receptor antagonist resulted in substantial disease improvement. Since skin manifestations are a common feature of AOSD, it should be among differential diagnoses in patients with skin lesions and constitutional symptoms. Biologic agents represent a significant therapeutic advance in patients with AOSD refractory to conventional therapy.

更新日期：2019-11-01

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