RESUMO
Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases. They may be revelatory of the disease or occur during follow-up. Antisynthetase syndrome (ASS) is a complex and heterogeneous autoimmune disorder. Antisynthetase antibodies, in particular the anti-Jo-1 antibody, characterize this syndrome. The occurrence and severity of ILD determine the prognosis, which in turn determines therapeutic management. We report the case of a 53-year-old female patient presenting with ILD, revealing the diagnosis of ASS. The evolution was favorable with bolus corticosteroids associated with cyclophosphamide.
RESUMO
El síndrome antisintetasa es una miopatía inflamatoria idiopática (MII) de origen autoinmune, poco frecuente, que se caracteriza por la presencia de autoanticuerpos antisintetasa ARNt (generalmente anti-Jo1), asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años de edad que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración del tratamiento con glucocorticoides asociado a metotrexato.
Anti-synthetase syndrome is a rare autoimmune inflammatory myopathy characterized by autoantibodies against tRNA synthetases (most commonly anti-Jo1) with clinical features that include myositis, interstitial lung disease, polyarthritis, mechanic's hands and Raynaud's phenomenon. We report a 45-year-old woman who presented with dermatomyositis phenotypical features and a significant improvement with corticosteroids and metotrexate treatment.