Collado María Victoria, Gargiulo María De Los Ángeles, Gómez Ramiro, Gómez Graciela, Pérez Nicolás, Suarez Lorena, Taratuto Ana Lía, Aruj Patricia
Sección Inmunología, Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires, Argentina. E-mail:
Sección Inmunología, Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires, Argentina.
Medicina (B Aires). 2018;78(5):360-363.
Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.
皮肌炎是一种具有多种临床表现的特发性炎性肌病。近年来,已鉴定出多种肌炎特异性抗体,包括抗MDA5抗体,该抗体对疾病的不同临床类型的诊断、预后及分类有帮助。这种抗体与皮肤溃疡、快速进展性间质性肺病、早期死亡率及不良预后相关,因此在合适的临床背景下检测到该抗体,就需要积极的免疫抑制治疗。我们描述了一例皮肌炎病例,分类为低肌病型(即轻度肌肉无力),有特异性皮肤病变、间质性肺病,且存在抗MDA5抗体,该病例对环磷酰胺、γ球蛋白及皮质类固醇联合治疗反应良好。